Plasma antiproteinase screen and neutrophil-mediated platelet activation. A major role played by alpha 1 antitrypsin.

Upon activation, human polymorphonuclear neutrophils (PMN) release two serine proteinases, cathepsin G (Cat.G) and elastase (HLE), which in turn synergize to activate nearby platelets. We looked for the inhibitory effect of plasma and the involvement of alpha 1 antichymotrypsin (alpha 1 ACT) and alpha 1 antitrypsin (alpha 1 AT), on this cell-to-cell cooperation. It was observed that inhibition by plasma of PMN-mediated platelet activation was rather correlated with an effect on HLE (r = 0.95) than on Cat.G (r = 0.65) enzymatic activity. Purified alpha 1 AT suppressed in a concentration-dependent manner HLE activity present in the supernatant of activated PMN. When HLE was fully blocked, alpha 1 AT started to inhibit Cat.G activity. By contrast and as expected, purified alpha 1 ACT inhibited only Cat.G activity. Using specific blocking polyclonal antibodies against alpha 1 AT and alpha 1 ACT, it was demonstrated that the inhibitory effect of plasma vs. HLE was entirely mediated by alpha 1 AT. By contrast, blockade of Cat.G activity was only partly due to plasma alpha 1 ACT and around 50% was attributable to alpha 1 AT. When plasma from patients with an acute inflammatory state was used in place of plasma from normal subjects, the inhibitory effect was more pronounced, while plasma depleted in alpha 1 AT and alpha 1 ACT was less effective. These data indicate a predominant role of alpha 1 AT in the inhibition by plasma of the PMN-mediated platelet activation.

Severe non-infectious circulatory shock related to hypopituitarism.

The authors report a case of refractory non-infectious circulatory shock with catecholamine and massive fluid loading-resistant features related to hypopituitarism. A 76-year-old man was admitted for shock after suffering from gastroenteritis for 3 days. He was pale and had sparse axillary and pubic hair and small testes. Right catheterization showed shock with low preload pressure and a low oxygen extraction ratio relevant for septic shock. Ultrasound tomography revealed a distended gallbladder due to a stone without peritoneal effusion. A non-inflammatory hydrops of the gallbladder was removed surgically. No microorganism was isolated. Cerebral computed tomography (CT) scan showed a pituitary mass. In the post-surgical period the shock became uncontrollable. Cortisol replacement therapy was instituted and clinical and hemodynamic improvement occurred after 2 h. Hormonal screening on admission before catecholamine administration showed a major decrease in all the hypothalamic-pituitary hormone concentrations. The patient died on day 15 with multiple organ failure. Hypopituitarism, probably owing to pituitary adenoma, was the only disease identified in this case. Hormone replacement therapy dramatically improved the clinical and hemodynamic status, although the role of an abdominal sepsis could not be eliminated. Arguments that pituitary hormone deficiency might increase the hemodynamic consequences of adrenal deficiency are discussed.

Emphysematous pyelonephritis related to specific gas-forming Escherichia coli without diabetes mellitus.

A 60-year-old-man without a history of diabetes mellitus, or invasive manipulation or obstruction of the urinary tract was admitted for septic shock. Type I emphysematous pyelonephritis was clear in this case: gas within the renal parenchyma extending into the subcapsular region and the perirenal space was present on spiral computerised tomography (CT). Surgical nephrectomy was performed because biochemistry, urography and CT identified a damaged non-functioning left kidney. The outcome was favourable. All urine, blood and nephrectomy specimen cultures were positive for a specific Escherichia coli which produced a high level of gas compared to a reference E. coli strain in the same standard medium, despite the absence of diabetes mellitus. Certain strains of bacteria are able to produce high levels of nitrogen, carbon dioxide and hydrogen and such fermentation in the absence of a high glucose serum level might explain the acute gas-producing bacterial renal infection.

Right-to-left interatrial shunt in ARDS: dramatic improvement in prone position.

The mechanisms leading to shunting through a patent foramen ovale include high right-sided cardiac pressures and respiratory factors due to mechanical ventilation and also anatomical changes in the right atrium as described in the platypnea-orthodeoxia syndrome. We report a patient with the adult respiratory distress syndrome (ARDS) who had a right-to-left atrial shunt which decreased in the prone position, after which oxygenation improved. The patient was admitted to the intensive care unit because of ARDS due to an invasive fungal infection. He had a history of chronic lymphocytic leukemia and paradoxical embolisms through a patent foramen ovale. Despite mechanical ventilation and antifungal treatment he developed severe ARDS. He was therefore turned to the prone position. Blood gas values improved dramatically (arterial oxygen tension/fractional inspired oxygen ratio increasing from 59 to 278 torr). Transcranial Doppler sonography was performed with bubble study, which confirmed a massive right-to-left shunt in the supine position and which instantaneously decreased in the prone position. This case suggests that a decrease in right-to-left shunt in patients who have a patent foramen ovale could partly explain the improvement in hypoxemia in the prone position.

[Cerebrospinal fluid complement and antinuclear antibodies in lupus meningoencephalitis]. / Complément et anticorps anti-nucléaires céphalo-rachidiens dans une méningo-encéphalite lupique.

Central nervous system involvement in systemic lupus erythematosus (SLE) requires immediate treatment. We report a case in a 30-year-old woman. Clinical features associated asthenia, headache, right nystagmus and coma. A mechanical ventilation was started. The neurologic pattern appeared three months after an initial treatment with pulsed doses of glucocorticoid (500 mg per day for 3 days) and one month after an oral cyclophosphamid regimen (50 mg twice a week). The cerebral involvement was evidenced by MRI and comparative analysis of the antinuclear auto antibodies (ANA) and the complement components in cerebral spinal fluid (CSF), pleural fluid and serum. The MRI slices showed a well-defined meningeal focal lesion. CSF-cell count was normal. CSF-proteins were elevated. ANA were positive, total complement (UI/l) was low, C4 component (g/l) was 0.11, undetectable and 0.25 respectively in plasma, CSF fluid and pleural fluid. The ANA specific pattern was anti-Sm2. We affirmed that specific cerebral injury was present because there were clinical and imaging features and a decrease of the C3 and C4 component in the CSF. The treatment associated in travenous pulsed doses of methylprednisolone (1000 mg per days for 3 days) and cyclophosphamid (500 mg per day for three days). Mechanical ventilation was with drawn one day after the end of the pulse therapy. The diminution of the complement component could help improving cerebral involvement of SLE. More clinical studies are required.

[Significance of single photon emission computed tomography and akinetic mutism]. / Intérêt de la tomoscintigraphie de perfusion pour le diagnostic de mutisme akinétique.

Akinetic mutism is a reactive status with permanent opening of the eyes. The accountable lesions are always bilateral. The injured cerebral structures include the frontal gyri, the thalami or the mesencephalic areas. In one case of a 44-year-old patient, magnetic resonance imaging and computed tomography were not contributive. Tc99m brain SPECT imaging was performed and displayed bilateral frontal hypoactivity. This case suggests that this technique could be helpful for diagnosis when clinical features and radiological pattern are opposite.

[Salicylism and glaucoma: reciprocal augmentation of the toxicity of acetazolamide and acetylsalicylic acid]. / Salicylisme et glaucome: augmentation réciproque de la toxicité de l'acétazolamide et de l'acide acétyl salicylique.

We report a case of salicylism caused by association of acetylsalicylic acid (ASA) and acetazolamide. A 50-year-old woman without chronic renal insufficiency, was admitted for confusion, cerebellar ataxia associated with hyperchloremic acidosis. She was treated with acetazolamide for simple glaucoma and since 14 days with ASA for acute pericarditis. Clinical features evoked salicylism despite low serum salicylate level: 178 mg/l. The carbonic anhydrase inhibitor explained the biochemical pattern. Acetazolamide-induced acidosis enhances the non ionized form of salicylate which crosses the biological membrane more rapidly and better than the ionized form. The tissular tropism of non ionized salicylate increases and cerebral toxicity may occur with low serum level of ASA. ASA also enhances the free form of acetazolamide.

[Non-hospitalization orders for subjects with presumed alcoholic intoxication]. / Le certificat de non hospitalisation des sujets présumés en état d'ivresse.

OBJECTIVES: French law requires all persons in an apparent state of alcoholic intoxication taken into police custody to be examined at hospital to determine whether medical observation is necessary. A do-not-hospitalize order is required to return the person to police custody. We attempted to ascertain the number of orders delivered and the quality of medical management of these persons. METHODS: Over a one-month period, 140 persons in an apparent state of alcoholic intoxication and under police custody were seen at the University Hospital emergency ward at Tours, France. Do-not-hospitalize orders were delivered for 131 of them who were returned to police custody. Among the 66 other persons admitted for acute intoxication, 4 were also returned to police custody. Nurse and physician reports were assessed. RESULTS: The 135 persons, accounting for 12.7% of all emergency ward activity, were predominantly men (96%) and young (mean age 33 years). Many were aggressive (12.7%) and 80% arrived between 6 p.m. and 6 a.m. Nurse records revealed care was less rigorous at night and for the more aggressive subjects. Reasons for delivery of the do-not-hospitalize order were not sufficiently explicit in the physician records. CONCLUSION: The large volume of activity involved in caring for these persons and the difficulty encountered in correct medical management, together with legal implications, suggest that medical and paramedical teams need better training for the management of persons in an apparent state of alcoholic intoxication.

[Adult bronchiectasis revealing familial ciliary anomaly]. / Dilatations des bronches localisées de l'adulte révélatrices d'une anomalie ciliaire familiale.

We report a case of bronchiectasis in a 26-year-old man associated with the following congenital abnormalities: deafness, purulent bronchorrhea, nasal polyps, dysmorphic physical pattern and chronic sinusitis. Situs inversus was absent. A sampling was performed on the posterior nasal mucous membrane and displayed structural ciliary abnormality: a deficiency of the intern dynein-arm. The patient's bother was affected and had similar features: congenital bronchiectasis, deafness, mental deficiency and sinusitis. Young's syndrome was relevant in this case. Hereditary ciliary dyskinesia should be considered in adults with bronchiectasis together with rhinologic and alimentary canal disorders. Nasal biopsies are safe and allow cilia examination.

[Losing consciousness: role of the venous lactate levels in the diagnosis of convulsive crises]. / Perte de connaissance: intérêt du dosage des lactates veineux pour le diagnostic de crise convulsive.

OBJECTIVES: This prospective study was conducted to evaluate the usefulness of venous lactate assay in the diagnosis of generalized seizures. PATIENTS AND METHODS: Over a three month period, 78 consecutive adults admitted to the emergency unit for unconsciousness were included in the study. Three study groups were defined: patients with generalized seizures (n = 22), unconscious patients without seizure (n = 34) and known epileptic patients with unexplained malaises (n = 22). Patients with a disease susceptible of increasing lactate levels were excluded. Peripheral venous blood was drawn to determine lactates, bicarbonates and pH on a blood gas analyzer. All determinations were performed within 5 minutes of blood withdrawal. CPK level was also determined with an enzymatic method. RESULTS: In patients who had seizures, venous lactate levels were higher than those in patients who had no seizures: 4.3 +/- 0.5 mmol/l in generalized seizure patients versus 1.64 +/- 0.1 and 2.2 +/- 1.39 in unconscious patients without seizure and known epileptic patients with unexplained malaise respectively. The threshold lactate level of 2.5 mmol/l given by ROC curves gave a 0.97 specificity and a 0.73 sensitivity. DISCUSSION: The acidosis observed in patients with generalized seizures results from the combined effects of respiratory and metabolic acidosis. High lactate level would be a consequence of hypoxemia, per seizure rise in catecholamines, and aerobic and anaerobic metabolism in muscles during the tonic-clonic phase. In patients presenting in an unconscious state, increased lactate levels, even when determined up to 2 hours after venous blood withdrawal, could be a useful parameter for the diagnosis of epileptic seizure.

[Septic shock with coma revealing typhoid fever]. / Choc septique avec coma révélateur d'une fièvre typhoïde.

BACKGROUND: Typhoid fever may be difficult to distinguish from malaria. Septic shock, encephalopathy and leukopenia are common features of both diseases. CASE REPORT: A 20-year-old South Korean woman was admitted to the intensive care unit with coma and shock. Vomiting and abdominal pain were followed by headache, prostration, fever and diarrhea. Leukocytopenia, lymphocytopenia, thrombocytopenia, rhabdomyolysis and hepatitis were present. Clotting tests were normal. The thick peripheral blood film was negative. Salmonella typhi was isolated from 6 blood cultures. Treatment associated ceftriaxone 4 g per day for 5 days, colloid and crystalloid fluids and dopamine. The patient was discharged 2 weeks later. DISCUSSION: Typhoid fever should be considered as a diagnosis in patients with sepsis who come from endemic zones. Abdominal symptoms, prolonged fever, coma and delayed headache are particularly contributive signs. Specific treatment should be instituted.

[Septic shock caused by Pasteurella multocida in alcoholic patients. Probable contamination of leg ulcers by the saliva of the domestic cats]. / Chocs septiques à Pasteurella multocida chez des patients alcooliques. Probable contamination d'ulcères de jambes par la salive de leur chat domestique.

BACKGROUND: Pasteurella multocida septicemic septic shock without scratches in chronic alcohol abusers are rare and paucibacillar. Chronic legs ulcers could be predisposing factors. Three severe cases of such diseases with early multiple organ failure without endocarditis despite 3, 5 and 3 positive blood cultures respectively are reported. CASE REPORTS: In all three cases, the source of septicemia was extensive skin legs ulcers contaminated by domestic cat saliva, probably. The underlying diseases were alcoholism, without cirrhosis (2 cases) and with cirrhosis (1 case). Aggravating factors were present (chronic renal insufficiency, diabetes mellitus, non-steroidal anti-inflammatory agents) and they might have been responsible for pejorative outcome despite early appropriate empirical antibiotic regimens. DISCUSSION: Because P. multocida SSC could be fulminant in chronic alcohol abusers with or without cirrhosis, we propose for these patients reducing exposure to cats and hand-washing after exposure if wounds are present.

-Severe self-poisoning with formol-. / Intoxication volontaire grave par le formol.

We report a case of voluntary poisoning with formalin in a 47-year-old man. The initial status included respiratory failure, metabolic acidosis and coagulopathy. Medical therapy consisted of mechanical ventilation, a single prolonged haemodialysis session, N-acetylcysteine, and folic acid administration. The corrosive damage to the gastrointestinal tract required an oesogastrectomy and three months later a colic transplant.

[Similarities between pleuropulmonary blastoma and Askin tumor]. / Similitude du blastome pleuropulmonaire de l'adulte et de la tumeur d'Askin.

Adult pleuropulmonary blastoma is an uncommon mesenchymal tumor of the peripheral lung parenchyma composed of undifferentiated cells. The differential diagnosis with Askin-Rosaï's tumor, an extrapulmonary pleuroparietal neuroectodermal tumor, may be a difficult task. We propose a similar therapeutic approach for these two malignant conditions: surgical excision associated with chemotherapy and autologous bone marrow transplant with adjuvant radiotherapy in case of recurrence. These two entities have many epidemiological, clinical, histological and immunohistochemical similarities.

[Pasteurella multocida pneumonia in a lupus patient: microbial identification with alveolar lavage fluid on blood culture medium]. / Pneumonie à Pasteurella multocida chez une patiente lupique. Identification microbiologique par l'ensemencement du liquide de lavage alvéolaire sur flacon à hémoculture.

BACKGROUND: Pasteurella multocida pneumonia mainly occurs in immunodepressed patients. Microbiological proof is difficult to obtain. CASE REPORT: A 36-year-old woman with systemic lupus erythematosus treated with cyclophosphamide and corticosteroids developed pneumonia. She was given amoxicillin-clavulanate. Bronchioalveolar lavage fluid cultures on gelose were negative but Pasteurella multocida grew on blood culture medium. DISCUSSION: Although the direct examination of bronchioalveolar lavage fluid demonstrated Gram negative coccobacilli, gelose cultures were negative, probably because of prior antibiotic therapy. The causal pathogen was only identified when BAL fluid was seeded on blood culture medium, allowing susceptibility tests and subsequent early adaptation of antibiotic therapy. This technique can be helpful in identifying the casual pathogen in microbial pneumonia.

[Weber-Rendu-Osler disease: pulmonary arterio-venous malformation with shunt disclosed after 5 occurrences of purulent meningococcal encephalitis]. / Maladie de Weber-Rendu-Osler: malformation artério-veineuse pulmonaire avec shunt révélé après 5 méningoencéphalites purulentes.

Hereditary hemorrhagic telangectasis or Weber-Rendu-Osler disease is associated with the presence of capillary malformations with pulmonary visceral shunts. These shunts are the cause of recurrent infections of the nervous system by loss of the anti-infectious lung filter. Hereditary hemorrhagic telangiectasis was diagnosed in a 68-year-old woman with a history of epistaxis, cutaneous telangectasis, purulent and pyogenic brain abscesses and meningitis. Outcome was favorable with antibiotic therapy. Ventilation as well as chest x-ray, brain scan and liver ultrasongraphy were normal. Blood gases showed a PO2 at 63 mmHg in ambient air and 62 mmHg with FiO2 = 1. There was no dyspnea or cyanosis nor any apparent polycythemia. Pulmonary angiography showed and arteriovenous malformation in the lower right lobe. Endovascular embolization was achieved with coils and N-butyl-cyano-acrylate glue which enabled angiographic occlusion and normalization of gas exchange: on ambient air the PaO2 = 71 mmHg and on FiO2 = 1, PaO2 was 359 mmHg. A true shunt was suspected on account of the association of hereditary hemorrhagic telangiectasis and recurrent meningitis. The diagnosis was suspected on blood gases alone.

[Microbial infection documented on admission to the intensive care unit for post-chemotherapy neutropenia after empiric antibiotic therapy]. / Infections microbiologiquement documentées à l'admission en réanimation pour neutropénie post-chimiothérapie après antibiothérapie probabiliste.

OBJECTIVE: The purpose of this study was to determine whether a systematic strategy of multiple microbiological samples for all adult patients with neutropenia admitted to an intensive care unit could document sepsis despite prior empiric antibiotic therapy. PATIENTS AND METHODS: All adults admitted to the intensive care unit with chemotherapy-induced neutropenia (WBC < 500/mm3) were included in the study. Microbiological samples were systematically obtained in all patients: 6 blood, 1 stool, 1 urine, 1 nasal swab. Data were analyzed for 54 consecutive patients: 22 men, 32 women, 42 with hematology diseases and 12 with solid tumors, mean age 46.6 +/- 14.4 years. Assessment of disease severity showed: simplified gravity index = 256 +/- 17; organ system failure score on day 1 = 2.42 +/- 1.1; 26 cases of septic shock among 47 cases of sepsis. Mortality was 50%. Within 48 h, bronchioalveolar lavage samples were obtained in 48 patients, and there were 12 spinal taps, 3 laparotomies, 3 skin biopsies and 6 sinus punctures. RESULTS: A microorganism was isolated in 39 patients (70%): 12 Gram negative, 12 Gram positive, 11 mycoses, 3 cases of herpes. Positive cultures were found for 31 blood, 1 bronchioalveolar lavage, 2 surgical, 5 serous and 22 oral or digestive tract samples. Documented microbial infection did not have prognostic value. Septic shock at admission was predictive of poor outcome. DISCUSSION: Despite prior antibiotic therapy in 46 patients, with a theoretically active drug in 37 cases, a systematic strategy of multiple deep samples increased the number of documented cases of infection in neutropenic patients. Oral and digestive tract infections predominated despite selective decontamination of the digestive tract.

[Amyotrophic lateral sclerosis manifesting as cognitive disorders. Value of brain perfusion scintigraphic tomography in intensive care]. / Sclérose latérale amyotrophique révélée par des troubles cognitifs. Intérêt de la tomoscintigraphie de perfusion cérébrale en réanimation.

BACKGROUND: Cognitive disorders have been described in amyotrophic lateral sclerosis, but usually after the diagnosis has ben established. CASE REPORT: A 57-year-old man was intubated for acute respiratory distress subsequent to pneumonia and diaphragm palsy. He had a 2-year history of drug-resistant depression and deterioration of cognitive functions. A pyramidal syndrome associated with biopsy-proven chronic neurogenic atrophy led to the diagnosis of amyotrophic lateral sclerosis. The electromyogram did not contribute to diagnosis. Brain MRI only evidenced moderate bilateral frontal-temporal atrophy. Brain SPECT demonstrated major perfusion defects in the frontal lobes. DISCUSSION: This patient had amyotrophic lateral sclerosis and frontal-temporal dementia with an unusually late onset clinical presentation: cognitive disorder was the inaugural sign. Brain SPECT and muscle biopsy enabled us to identify the cortical and peripheral motor neurone involvement in this uncooperative intensive care patient totally dependent on mechanical ventilation.

[Fatal toxic respiratory epitheliolysis. Subacute tracheo-bronchial desquamation in Stevens-Johnson syndrome]. / Epithéliolyse respiratoire toxique fatale. Desquamation suraiguë trachéo-bronchique lors d'un syndrome de Stevens-Johnson.

Acute bronchial mucosal sloughing related to Toxic Epidermal Necrolysis (Lyell syndrome) is widely reported in literature. On the contrary severe respiratory involvement is rare in post-infectious or toxic Epitheliolysis (Stevens-Johnson syndrome). There is no well-known predictive sign of bronchial epithelium involvement. An 18-year-old patient was admitted for Stevens-Johnson syndrome related to sulfasalazine (salazosulfapyridine). There were no respiratory signs. An acute respiratory failure occurred 36 hours after from admission due to an obstructive and desquamative necrosis of the tracheobronchial epithelium. We purpose that a fiberoptic laryngoscopy should be performed even in non-dyspneic patients suffering from Stevens-Johnson syndrome if hypersecretion is present. Fiberoptic bronchoscopy can be helpful in these cases.

[Beginning rheumatoid arthritis revealed during bronchiectasis surinfections. Value of cyclic antibiotherapy?]. / Polyarthrite rhumatoïde débutante révélée au décours de surinfections de bronchectasies. Place d'une antbiothérapie cyclique?

Incidence of symptomatic bronchiectasis (BR) occurs in around 2% in patients with late rheumatoid arthritis (RA). Its seems that the association BR-RA could be a worsening factor for outcome of RA patients. A 58-year-old woman without dry syndrome, suffering from bronchial purulence over one year was admitted to the Department of Pneumology for hemoptysis and arthritis (knees, ankles, and wrists). Three prior episodes of inflammatory articular pain had occurred after transient bronchial purulence or pneumonitis. CT-scan showed bilateral bronchiectasis. Diagnosis of early RA was proved after the third episode of bronchial purulence related to a strain of Haemophilus influenzae. A strain of Coxiella burnetii was probably responsible for one of the three bronchial surinfections. Latex and Waaler Rose tests were transiently positive during the first episode, and became positive after the third one. At that time, RA was relevant in view of ARA criteria. Cyclic prophylactic antibiotic regimens could be proposed to patients suffering from RA-BR association, in contrast to the cases of patients with isolated BR. This approach could prevent destabilization of RA and reinforce of anti-rheumatic therapy. Activation and release of cytokines (NFk-B, TNF-alpha), and/or bacterial epitopes seems to be directly responsible for the articular destabilization.