[Autoimmune hemolytic anemia in children]. / Anémie hémolytique auto-immune de l'enfant.

Autoimmune hemolytic anemia is a rare condition in children which differs from the adult form. It is defined by immune-mediated destruction of red blood cells caused by autoantibodies. Characteristics of the autoantibodies are responsible for the various clinical entities. Classifications of autoimmune hemolytic anemia include warm autoimmune hemolytic anemia, cold autoimmune hemolytic anemia, and paroxysmal cold hemoglobinuria. For each classification, this review discusses the epidemiology, etiology, clinical presentation, laboratory evaluation, and treatment options.

Behavioral and neurophysiological study of attentional and inhibitory processes in ADHD-combined and control children.

This study compares behavioral and electrophysiological (P300) responses recorded in a cued continuous performance task (CPT-AX) performed by children with attention deficit hyperactivity disorder-combined subtype (ADHD-com) and age-matched healthy controls. P300 cognitive-evoked potentials and behavioral data were recorded in eight children with ADHD (without comorbidity) and nine control children aged 8-12 years while performing a CPT-AX task. Such task enables to examine several kinds of false alarms and three different kinds of P300 responses: the "Cue P300", the "Go P300" and the "NoGo P300", respectively, associated with preparatory processing/attentional orienting, motor/response execution and motor/response inhibition. Whereas hit rates were about 95% in each group, ADHD children made significantly more false alarm responses (inattention- and inhibition-related) than control children. ADHD children had a marginally smaller Cue P300 than the control children. Behavioral and electrophysiological findings both highlighted inhibition and attention deficits in ADHD-com children in the CPT-AX task. A rarely studied kind of false alarm, the "Other" FA, seems to be a sensitive FA to take into account, even if its interpretation remains unclear.

[Neurological soft signs in schizophrenic patients and their nonaffected siblings]. / Signes neurologiques mineurs chez les patients schizophrènes et leurs apparentés non malades.

BACKGROUND: Neurological soft signs (NSS) are subtle neurological signs indicating non specific cerebral dysfunction. Several studies have found an excess of NSS in schizophrenic patients compared to healthy subjects. Although NSS have been consistently reported in schizophrenic patients, their clinical relevance and their relation to functional impairment and severity of this disease are not well-clarified. In addition, the presence of NSS in schizophrenic patient's relatives suggests that they could be associated with the genetic liability. OBJECTIVES: To determine the prevalence and scores of NSS in schizophrenic patients and their nonaffected siblings and to examine the clinical correlates of NSS in the schizophrenic patients. METHOD: Sixty-six schizophrenic patients (50 males and 16 females, mean age=31.16+/-7.17 years), were compared to 31 of their nonaffected siblings (22 males and nine females, mean age=32.19+/-5.88 years) and to 60 controls subjects (40 males and 20 females, mean age=30.70+/-6.54 years) without family psychiatric history. NSS were assessed with Krebs et al.'s neurological soft signs scale. It is a comprehensive and standardized scale consisting of 23 items comporting five factors: motor coordination, motor integration, sensory integration, quality of lateralization and involuntary movements or posture. The Simpson and Angus scale for extrapyramidal symptoms was also rated. Clinical assessment of the schizophrenic patients was conducted using the positive and negative syndrome scale (PANSS), clinical global impressions (CGI) and global functioning evaluation (GAF). Psychiatric disorders were ruled out among siblings of schizophrenic patients and control subjects by psychiatric review evaluation, according to the DSM-IV check list. RESULTS: When the total NSS score of 11.5 was considered the cut-off point, the prevalence of NSS was 96.9% in the schizophrenic patients versus 35.5% in the nonaffected siblings (p<0.0001). Schizophrenic patients had also significantly higher NSS total score and subscores than the siblings and control groups. The NSS total score was 19.51+/-5.28 in the schizophrenic patients, 10.77+/-3.38 in their nonaffected siblings and 4.23+/-2.07 in control subjects (p<0.0001). The NSS total score and subscores in the siblings group were intermediate between those of the schizophrenic patients and those of the control subjects. The motor coordination, motor integration and sensory integration subscores were higher in schizophrenic patients and their nonaffected siblings. The NSS total score correlated positively with the negative (p<0.0001) and disorganization symptoms (p=0.001) subscores and total score of PANSS (p=0.004). The PANSS total score and negative and disorganization subscores also correlated positively with the motor integration and quality of laterality subscores of NSS. The NSS total score was significantly correlated with severity of illness (p<0.0001), lower educational level (p=0.002) and poor global functioning (p=0.003). CONCLUSIONS: The association between NSS with negative and disorganization dimensions of schizophrenia supports that neurological dysfunction is an intrinsic characteristic of the illness and may distinguish a subgroup of patients with poor illness course and outcome. The NSS could be a trait marker useful in phenotypic characterization of schizophrenic patients and identification of vulnerability in genetically high-risk subjects.

Allogeneic hematopoietic stem cell transplantation in Tunisia.

In 1998, the Tunisian team of the 'Centre National de Greffe de Moelle Osseuse' initiated allogeneic hematopoietic SCT (AHSCT) in Tunisia. As of June 2007, information was collected about 299 patients with a first AHSCT and 12 additional retransplants. The median age was 19 years (range 2-49 years). The main indications were aplastic anemia (n=106, 36%), leukemia and nonmalignant disorders (n=153, 51%), Fanconi anemia (n=26, 9%) and other nonmalignant disorders (n=14, 4%). Preparative regimens depended on indication. All donors were HLA geno-identical. The stem cell sources were BM (87%) and PBSCs (13%). At the time of analysis, 200 patients (67%) were alive after a median follow-up of 42 months (range 3-112 months). The overall TRM rate was 17%. Outcome depended on indication. According to our results, allogeneic HSCT is potentially curative for hematological diseases, but it is a toxic approach for malignant disorders.

Prospective randomised comparison of the COBE spectra version 6 and haemonetics MCS(+) cell separators for hematopoietic progenitor cells leucapheresis in patients with multiple myeloma.

A randomised crossover trial of two separators was undertaken to compare the mononuclear cell, CD34(+) cell and CFU-GM yield, in patients (<61 years) with previously untreated symptomatic multiple myeloma. After first-line therapy, all patients received mobilising chemotherapy (cyclophosphamide 4 g/m(2)) and daily G-CSF. The first leucapheresis was performed on the first day the peripheral blood absolute CD34(+) cell count was > 20 cells/microl. All patients underwent 2 leucaphereses on consecutive days. The patients were randomised to undergo either the first or second leucapheresis using the COBE Spectra. The target duration of the procedure on the COBE Spectra was 2 total blood volumes, and for the Haemonetics MCS(+) it was 20 cycles with four recirculations. Between September 2003 and March 2005, 60 patients were entered in the study. COBE Spectra version 6 processed significantly larger volumes of blood than the Haemonetics MCS(+) (8,845 and 5,680 ml, respectively, P < 0.01). The absolute yield of mononuclear cells (2.1 vs. 1.5 x 10(8)/kg, P = 0.04), CFU-GM (11 vs. 3 x 10(4)/kg, P = 0.01) and CD34(+) cells (3 vs. 1.7 x 10(6)/kg, P = 0.02) were all significantly higher with the COBE Spectra version 6, as were the yields per unit volume of blood processed. In conclusion, our study shows that COBE Spectra Version 6 is faster and has a better yield than the Haemonetics MCS(+), in patients with multiple myeloma.

Frontotemporal dementia progresses to death faster than Alzheimer disease.

BACKGROUND: Frontotemporal lobar degeneration (FTLD) is a common cause of non-Alzheimer dementia, but its natural history and the factors related to mortality in affected patients are not well understood. METHODS: This retrospective, longitudinal study compared survival in FTLD (n = 177) with Alzheimer disease (AD; n = 395). Hazards analysis investigated the contribution of various demographic, neuropsychiatric, and neuropsychological variables and associated neurologic and neuropathologic findings. RESULTS: The frontotemporal dementia (FTD) subtype of FTLD progressed faster than AD (median survival from retrospectively determined symptom onset, 8.7 +/- 1.2 vs 11.8 +/- 0.6 years, p < 0.0001; median survival from initial clinic presentation, 3.0 +/- 0.5 vs 5.7 +/- 0.1 years, p < 0.0001). Survival was similarly reduced in the related conditions corticobasal degeneration and progressive supranuclear palsy. Survival in the semantic dementia subtype of FTLD (11.9 +/- 0.2 years from onset and 5.3 +/- 0.4 years from presentation), however, was significantly longer than in FTD and did not differ from AD. Hazards analysis to determine factors affecting survival in FTLD showed no effect of age at onset, sex, education, family history, or neuropsychiatric profile. Among neuropsychological measures examined, impaired letter fluency had a significant association with reduced survival. Associated ALS significantly reduced survival in FTLD. The presence of tau-positive inclusions was associated with the slowest progression. CONCLUSIONS: Frontotemporal lobar degeneration progresses more rapidly than Alzheimer disease, and the fastest-progressing cases are those with the frontotemporal dementia clinical subtype, coexisting motor neuron disease, or tau-negative neuropathology.

The French Addenbrooke's cognitive examination is effective in detecting dementia in a French-speaking population.

We evaluated the sensitivity and specificity of our French version of Addenbrooke's cognitive examination (ACE) to detect dementia in our patient population. One hundred and fifty-eight cases were included in the study. In our patient series, the sensitivity for diagnosing dementia with a Mini-Mental State Examination (MMSE) score of < or = 24/30 was 48.5%, the sensitivity of an MMSE score of < or = 27/30 was 82.5% with a specificity of 72.1%, the sensitivity of an ACE score of < or = 83/100 was 86.6% with a specificity of 70.5% and the sensitivity of an ACE score of < or = 88/100 was 97.9% with a specificity of 59%. We conclude that the French version of the ACE is a very accurate test for the detection of dementia, and should be widely used in clinical practice.

Is the Addenbrooke's cognitive examination effective to detect frontotemporal dementia?

We evaluated the Addenbrooke's cognitive examination (ACE), a simple instrument to differentiate frontotemporal dementia (FTD) from Alzheimer's disease (AD), in our dementia patients clinic population. The Verbal-Language/Orientation-Memory (VLOM) ratio, which compares its language and memory scores, determines whether FTD or AD is more likely. The ACE was translated into French with adaptation maintaining the number of words in the name and address learning and delayed recall test, and with cultural adaptation for the semantic memory. The 85 included subjects had no evidence of two or more organic pathologies, after at least six months of follow-up, and an MMSE score>or=20/30. Patients with cognitive impairment due to alcohol intake were excluded. The diagnosis of a specific dementing illness was based on the consensus of the neurologist and neuropsychologists in the team. Thereafter, another neurologist expert in dementia, blinded to the ACE result and to the diagnosis and treatment, reviewed all cases files and proposed a diagnosis. A diagnostic agreement was reached for 79 cases (92.9%) with 40 (50.6%) dementia: 25 AD (62.5 %), 9 FTD (22.5 %). We estimated that the sensitivity for detecting dementia of an ACE score3.2 was 72%,with a specificity of 69.4%. We conclude that, when used as originally proposed, ACE is very accurate for the detection of dementia, but much less effective in discriminating the most common frontal variant of FTD.

Control of in vitro prostaglandin F2 alpha and E2 synthesis by caruncular and allantochorionic tissues from cows that calved normally and those with retained fetal membranes.

High concentrations of PGF2 alpha and PGE2 are produced by the uterus during the early postpartum period in cows and may play an important role in both placental separation and uterine involution. In the present study, we have examined the hormonal and intracellular control mechanisms involved in PGF2 alpha and PGE2 secretion by caruncular and allantochorionic tissue in vitro. Tissue explants, obtained about 6 hr postpartum from cows that delivered normally (NFM, n = 10) or cows with retained fetal membranes (RFM, n = 4), were incubated for 6 hr and PGF2 alpha and PGE2 concentrations in the medium were determined by radioimmunoassay. Addition of oxytocin (100 microU/ml), platelet activating factor (PAF, 100 ng/ml) and epidermal growth factor (EGF, 100 ng/ml) had no effect on secretion of PGF2 alpha from the caruncle, but oxytocin and PAF did stimulate PGE2. There was no difference between groups of cows. All three substances stimulated PGF2 alpha from the allantochorion of NFM, but not RFM, cows and stimulated PGE2 secretion from the allantochorion of both groups of cows. Incubation of the tissues with cholera toxin (100 ng/ml), dibutyryl cyclic adenosine 3',5'-monophosphate (dibutyryl cAMP, 1 mM), calcium ionophore A23187 (5 microM) or phorbol ester 12-myristate-13 acetate (PMA, 100 nM) showed that PGF2 alpha secretion is essentially via the calcium-protein kinase C effector pathway. However, calcium-protein kinase C and cAMP second messenger systems appear to be involved in the secretion of PGE2. Prostaglandin secretion was sensitive to cycloheximide in both caruncular and allantochorionic tissues, suggesting that protein synthesis may be involved. In conclusion, these data show that in vitro PGF2 alpha secretion can be modulated by the agonists used only in allantochorion and is essentially via the calcium-protein kinase C effector pathway. PGE2 secretion can be modified in both caruncular and allantochorion tissues and involves both inositol triphosphate-diacylglycerol and cAMP second messenger systems.

Effect of bacterial cell wall and lipopolysaccharide on arachidonic acid metabolism by caruncular and allantochorionic tissues from cows that calved normally and those that retained fetal membranes.

Immunoactive eicosanoids may have a role in both placental separation and uterine involution in cattle. In the present study, we examined the effects of bacterial cell wall preparation and endotoxins on in vitro prostaglandin synthesis and arachidonic acid (AA) metabolism by caruncular and allantochorionic tissues. Placentomes were obtained about 6 h post partum from cows that delivered normally (n = 10) or those with retained fetal membranes (n = 4); the tissue explants were incubated for 6 h in the presence of labeled or nonlabeled AA. Prostaglandin F(2alpha) (PGF(2alpha)) and E(2) (PGE(2)) were measured by radioimmunoassay, and labeled AA metabolites were separated by reverse phase-high pressure-liquid chromatography. There was no effect of bacterial cell wall preparations or endotoxins on in vitro caruncular PGF(2alpha) secretion. However, bacterial products increased caruncular PGE(2) secretion in both cows that delivered normally and those with retained fetal membranes. For normal delivery cows caruncular tissue, bacterial product also increased leukotriene B(4) (LTB(4)) and decreased both thromboxane B(2) (TXB(2)) and hydroxy-eicosatetranoic acids (HETE) in vitro secretion. For the allantochorion, bacterial products increased in vitro PGF(2alpha) secretion only in cows that delivered normally and increased PGE(2) secretion essentially in cows with retained fetal membranes. In general, 6 keto PGF(1alpha) was the main metabolite secreted by both allantochorionic and carucular tissues. However, in cows with retained fetal membranes, PGE(2) became the most important metabolite secreted by allantochorion, especially in the presence of endotoxin. In conclusion, these results suggest that bacteria found in the early postpartum uterus or their endotoxin affect primarily caruncular and allantochorionic PGE(2) synthesis.

[Quantitative assessment of the quality of life of patients with a kidney transplant]. / Quantitative Erfassung der Lebensqualität bei Patienten mit einer Nierenersatztherapie.

Actually three methods are offered for the treatment of patients with chronic renal failure: 1. Hemodialysis, 2. chronic peritoneal dialysis for outpatients (CAPD) and 3. Transplantation of a kidney. In order to define the optimal therapy for these patients not only economic factors but also quantitative evaluation of life quality has to be taken into account. To this end a questionnaire called "Sickness Impact Profile", that covers most of the relevant features of life and is well established in the Anglo-Saxon countries, was translated into German language and tested in 34 hemodialysis-patients, 25 CAPD-patients and 29 patients with kidney transplant, in order to investigate the applicability. Results showed that the German issue can successfully be applied to Swiss patients with renal failure and lead to the conclusion that--in accordance to results of similar studies in the USA--life quality in patients with kidney transplant is better than in patients treated with hemodialysis or CAPD.

Leukotriene B4 in cows with normal calving, and in cows with retained fetal membranes and/or uterine subinvolution.

Two experiments were performed to study the relationship between leukotriene B4 (LTB4) synthesis and placental separation and uterine involution in the cow. In experiment I, the concentration and synthesis of LTB4 by caruncular tissue was lower in cows with retained fetal membranes (RFM cows, n = 11) than in cows that expelled the fetal membranes normally (NFM cows, n = 19). The presence of bacterial cell wall, especially of alpha-hemolytic streptococci and coagulase positive staphylococci enhanced LTB4 synthesis by allantochorion only in NFM cows. In the RFM group, Escherichia coli lipopolysaccharide decreased allantochorionic LTB4 synthesis. With caruncle, only epidermal growth factor increased LTB4 production in NFM cows. In experiment II, the caruncular and endometrial secretion of LTB4 was lower in cows with subuterine involution (SUI cows, n = 5) or cows with SUI and RFM (SUI+RFM cows, n = 4) than in cows with normal uterine involution (NUI cows, n = 8). This decrease was especially noticeable in the previously gravid horn. In the three uterine involution groups, there were no differences in LTB4 synthesis by caruncular tissue taken from the previously gravid horn. However, progesterone and a bacterial suspension of E. coli reduced the synthesis of LTB4. Estradiol had no effect on LTB4 synthesis at the end of the postpartum period. These results suggest that LTB4 may play an important role in both placental separation and uterine involution in cattle and LTB4 synthesis may be modulated by endocrine and bacterial factors.

Metabolism of arachidonic acid by caruncular and allantochorionic tissues in cows with retained fetal membranes (RFM).

The metabolism of arachidonic acid (AA) by caruncular and allantochorionic tissues and its regulation was studied in normal cows (n = 13) and those with retained fetal membranes (RFM; n = 9). Tissues were taken via the vagina about 6 hours postpartum and incubated for 6 hours in minimum essential medium containing tritiated AA alone or in the presence of oxytocin, platelet activating factor (PAF), epidermal growth factor (EGF) or ionophore calcium (A23187). The metabolites of AA were separated by reverse phase-high pressure-liquid chromatography. Tissue concentrations of prostaglandin F2 alpha (PGF2 alpha) and prostaglandin E2 (PGE2) and plasma 13,14-dihydro-15-keto-PGF2 alpha (PGFM) concentration were also measured by radioimmunoassay. For caruncular tissue, less thromboxane B2 (TXB2) and more 6-keto prostaglandin F1 alpha (PGIM) was synthesized in tissue from the animals with RFM than in the controls. Oxytocin, PAF, EGF and A23187 increased only PGIM production in the control animals; A23187 also decreased TBX2 synthesis. For the allantochorion, more PGE2, leukotriene B4 (LTB4) and PGIM and less TXB2, PGF2 alpha and hydroxyecosatetranoic acids (HETE) was synthesized in tissue from cows with RFM than from animals that delivered normally. All of the substances used in this study increased PGIM, PGF2 alpha and LTB4 and decreased TXB2 production by the allantochorionic tissue in control animals. The metabolism of AA by the allantochorionic tissue seems quantitatively under hormonal control. The metabolism of AA at the level of both maternal and fetal components of the placenta in cows with RFM differed from that seen in animals that expelled the membranes normally.

[Epidemiologic study of anti-HCV antibodies in Tunisian blood donors]. / Etude épidémiologique des anticorps anti-HCV chez les donneurs de sang en Tunisie.

A study carried out on 2,006 Tunisian blood donors permitted us to evaluate the prevalence of hepatitis C virus antibodies (anti-HCV) which was 1.09%; frequency of indirect markers of NANBH were as follows: ALAT greater than 2 N (1.24%), anti-HBc (30.35%), 55.5% of HCV (+) donors had anti-HBc (+), 12.5% had rises ALAT and only 33.3% both markers.

A versatile hydraulically operated respiratory servo system for ventilation and lung function testing.

A description is given of the design and performance of a microcomputer-controlled respiratory servo system that incorporates the characteristics of a mechanical ventilator and also allows the performance of a multitude of test procedures required for assessment of pulmonary function in paralyzed animals. The device consists of a hydraulically operated cylinder-piston assembly and solenoid valves that direct inspiratory and expiratory gas flow and also enable switching to different test gas sources. The system operates as a volume-flow-preset ventilator but may be switched to other operational cycling modes. Gas flow rates may be constant or variable. The system operates as an assister-controller and, combined with a gas analyzer, can function as a "demand" ventilator allowing for set-point control of end-tidal PCO2 and PO2. Complex breathing maneuvers for a variety of single- and multiple-breath lung function tests are automatically performed. Because of the flexibility in selection and timing of respiratory parameters, the system is particularly suitable for respiratory gas studies.