Renal and adrenal gland localization of chronic myelomonocytic leukemia presenting as a kidney tumor.

Chronic myelomonocytic leukemia (CMML) is a myelodysplastic syndrome (MDS) characterized by prominent monocytosis and an increase in bone marrow monocyte precursors in addition to dyshaematopoietic features (1). Extrahaematological manifestations including cutaneous, neurologic, and rheumatic symptoms have been recorded in association with CMML. Here, we report the first observation of renal, adrenal and perirenal involvement in CMML which presented as a kidney tumor.

Short-term impact of the European Consensus Conference on the use of selective decontamination of the digestive tract with antibiotics in ICU patients.

Because it remained controversial, the use of selective digestive decontamination (SDD) in patients in the intensive care unit (ICU) was chosen as the topic of the first European Consensus Conference in Intensive Care Medicine (ECCICM) in December, 1991. The Consensus Bureau decided to assess the impact of this conference 2 years afterwards. For this purpose, a questionnaire was sent to the members of the European Society of Intensive Care Medicine, the Societé de Réanimation de Langue Française and the Societé Française d'Anesthesie et Réanimation before the conference. The recommendations following the conference discouraged the systematic use of SDD in ventilated patients and urged the monitoring of bacterial resistance and adapting antibiotics to epidemiology of the units. Two years after the conference, the same questionnaire was sent to those physicians who had responded to the first one. Eighteen percent used SDD for all ventilated patients and 17% remain users after 2 years. Among the occasional (32%) or continual (17%) users of SDD, the regimens used were mostly intravenous cefotaxime (60% of systemic antibiotics) and a topical combination of polymixin E, tobramycin, and amphotericin B (62% of overall topical combinations). The antibiotics used were unchanged after 2 years in almost all cases. In conclusion, the short-term impact of the Consensus Conference on SDD in ICU patients has been poor. This may be related to the continuing insufficiency of strong, definite data regarding the impact of this technique upon mortality and the theoretical risk of resistance to antibiotics, thus allowing physicians to stick to their policies until there is new evidence.

[Lung disease induced by isotretinoin]. / Pneumopathie induite par l'isotrétinoïne.

A young man without any past history of note had taken isotretinoin for disfiguring acne before the summer season. He presented with a severe bilateral pneumonia, associated with dyspnoea two months after the start of treatment. On the pulmonary radiography there was a bilateral ground glass appearance which was worse on the right. The elevated level of eosinophils (54% in 564,000 cells/ml) in the alveolar lavage lead to a diagnosis of allergic pneumonia. The rapidly favourable outcome following the cessation of the medication and with the addition of corticosteroids seemed to us a supplementary argument in favour of a diagnosis of eosinophilic pneumonia, due to isotretinoin which seemed the primary initiating factor.

[Visceral form of human anthrax imported from Africa]. / Forme viscérale de charbon humain importée d'Afrique.

Widespread vaccination has largely eliminated anthrax in Europe (the last case was reported in France in 1972) but the disease remains endemic in many developing countries. The usual cutaneous presentation (malignant pustules) is much more familiar than the various visceral manifestations including digestive tract, pulmonary or meningeal signs. We report a case of a 33-year-old immigrant living in France who was hospitalized for asthenia, dyspnoea, mucopurulant expectoration and moderate diarrhoea 3 days after a 3-month stay in Senegal and Gambia. The temperature was 39 degrees C at admission and blood pressure 110/70 mmHg. Crepitants were heard at the base of the right lung and the rest of the physical examination was normal. Blood was drawn for culture. Laboratory tests and the chest X-ray led to the diagnosis of pneumopathy and a treatment of amoxicillin and clavulanic acid was given with oxygenotherapy. The patient's temperature returned to normal but over the next 48 hours the dyspnoea worsened together with the black diarrhoea. The abdomen was painful. There were no skin lesions. The chest X-ray revealed an extension of the bilateral pulmonary images and bilateral pleural effusion. Laboratory tests revealed thrombopenia (platelet count 38,000/mm3) hyperleukocytosis (WBC 48,000/mm3) and haemolysis (Hb 4 milligrams). The diagnosis was made on the basis of the initial blood cultures which were positive for Bacillus anthracis. All other samples were negative, including HIV serology. Despite adapted antibiotic therapy (penicillin G, 8MU/day, was initiated on day 2), multiple organ failure occurred with septic shock and pulmonary oedema. The patient died in the intensive care unit on day 7. Fatal outcome due to anthrax is described in 25% of the visceral forms but reaches 100% in cases of septicaemia. The haemolysis observed in this case is not mentioned in the classical descriptions of anthrax. When treating septic syndromes in patients who have returned from endemic zones, clinicians should entertain the diagnosis of anthrax since the risk of fatal outcome is increased greatly in case of delayed diagnosis.

Ultralow doses of vasopressin in the management of diabetes insipidus.

Management of postoperative diabetes insipidus (DI) frequently requires intermittent treatment with multiple subcutaneous injections of pituitrin or vasopressin, in doses averaging 20 IU/24 h. Use of a syringe pump for a continuous infusion of ultralow doses of pituitrin produced uniform, constant, and sustained reduction of urinary output, thus facilitating regular fluid replacement. Twelve patients with postoperative DI received iv pituitrin at a dose of 1.6 +/- 0.26 mIU/kg X h (1 to 2 IU/24 h). The antidiuretic effect began at the third hour of treatment, peaked by the sixth hour (diuresis of 37 ml/h, specific gravity of 1.018 +/- 0.002), and was sustained throughout infusion. Polyuria recurred 3 h after the infusion was discontinued; this rapid reversibility is highly advantageous when excessive fluid intake causes overhydration. Pressor effects were not observed during the treatment period.

[Hypovolemic shock caused by increased capillary permeability associated with monoclonal gammopathy. New case and review of the literature]. / Choc hypovolémique par augmentation de la perméabilité capillaire associé à une gammapathie monoclonale. Nouvelle observation et revue de la littérature.

The authors report another case of hypovolaemic shock due to increased capillary permeability and review previous reports in the medical literature. It is a rare condition with all patients presenting hypovolaemic shock of sudden onset with varying degrees of hypoproteinaemia and increased haematocrit. A monoclonal globulin peak is usually observed on serum electrophoresis during and between episodes of shock. The pathological role of this globulin in the increased capillary permeability has not been established.

[Hypokalemic paralysis after prolonged absorption of a non-alcoholic pastis in a diabetic]. / Paralysie hypokaliémique après absorption prolongée d'un pastis sans alcool chez un diabétique.

A typical case of glycyrrhizin-induced hypokalemic paralysis in a diabetic patient is presented. Hypokalemia was responsible for a generalized muscle weakness with a rise in serum muscular enzymes and cardiovascular disorders, which improved under potassium supplementation and spironolactone therapy. The pseudoaldosteronism syndrome with low plasma renin activity and aldosteron suggested aldosteron -like liquorice intoxication. Patient's interrogation revealed regular consumption of a common aperitive drink: a non-alcoholic pastis containing small amounts of glycyrrhizin. The discrepancy between the severity of clinical manifestations and the ingested amounts of glycyrrhizin is explained by the diabetic state.

Dantrolene--a new therapeutic approach to the neuroleptic malignant syndrome.

The Neuroleptic Malignant Syndrome (NMS) is a rare but severe affection (spontaneous mortality 30 to 50 per cent), associating fever, hypertonia with myolysis, and respiratory impairment. Its mechanism remains debatable: The origin of the hypertonia might be central (as phenothiazines and butyrophenones induce a blockade of dopaminergic receptors in the hypothalamus) or it might be muscular (with an impairment of the sarcoplasmic reticulum uptake of calcium in a genetically abnormal muscle, as is proven in malignant hyperthermia). Whatever the actual mechanism, the oral or intravenous administration of sodium dantrolene, a peripheral muscle relaxant agent which does not affect the neuromuscular transmission but prevents the calcium-dependent contraction of actin and myosine, has proved to be effective in three recent cases of NMS.

[Long-term effects of plasma exchange in myasthenia. Results of a randomized study]. / Effets à long terme des échanges plasmatiques au cours de la myasthénie. Résultats d'une étude randomisée.

A randomized study was carried out in 14 myasthenia patients to compare the long-term effects of two therapeutic regimens. Group I patients received prednisone 1 mg/kg/24 h for one month, then in decreasing dosage; in case of failure at the end of the first month, cyclophosphamide 2 mg/kg/24 h was added to the prednisone treatment. Group II patients received the same treatment associated with 3 plasma exchanges over a 10-day period; these were continued, if required, at the rate of once a week. The minimum follow up was one year. The results (greater improvement in muscular strength and vital capacity) were better after one month in group I and thereafter similar in both groups. However, the mean daily dose of prednisone was higher in Group I. The number of exacerbations of myasthenia was greater in group II (11 versus 2 in group I over a 24-months period). The mean fall in anti-R Ach ab was about the same in both groups. This study confirms the rapid effectiveness of plasma exchanges and their value in severe myasthenia. The higher incidence of exacerbations in Group II was probably due to a rebound phenomenon and points to the need for combined immuno-suppressive treatment.

Beneficial effects of dantrolene in the treatment of neuroleptic malignant syndrome: a report of two cases.

Two schizophrenic patients developed muscular rigidity, stupor, and hyperpyrexia consistent with neuroleptic malignant syndrome, 8 to 10 days after starting haloperidol therapy. Muscle rigidity was not affected by etybenzatropine or diazepam, but dantrolene, a direct-acting skeletal muscle relaxant, provided muscle relaxation with a concomitant decrease of fever and serum creatine kinase. Neuroleptic malignant syndrome and malignant hyperthermia are clinically similar, and dantrolene is effective in both; suggesting a muscular origin of fever in these two diseases.

[Idiopathic orthostatic hypotension and the Shy-Drager syndrome: hemodynamic and pharmacological studies in seven patients (author's transl)]. / Hypotension orthostatique idiopathique et syndrome de Shy-Drager. Etudes hémodynamiques et pharmacodynamiques chez sept sujets. Limites nosologiques.

Idiopathic orthostatic hypotension (IOH) is a slowly progressive disease of the autonomic nervous system. The association of central lesions with those of IOH is characteristic of the Shy-Drager syndrome, which follows a rapidly unfavorable course, and can be distinguished from IOH, according to some authors, by anatomical and pharmacological features. Hemodynamic and pharmacological studies were conducted in 4 cases of IOH and 3 cases of the Shy-Drager syndrome in order to determine the localization and extent of the orthostatic regulatory disorder. No differences were found between the two groups and a common physiopathological mechanism for the two affections is suggested.