Hemophagocytic lymphohistiocytic syndrome: Unrecognized cause of multiple organ failure.

OBJECTIVE: To describe an often-unrecognized clinical picture of multiple organ failure in hemophagocytic lymphohistiocytic syndrome (HLS). DESIGN: Retrospective chart review. SETTING: A ten-bed pediatric intensive care unit (PICU) in a tertiary children's university hospital. PATIENTS: A total of 11 children (age, 5 months to 13 yrs) who fulfilled the criteria for the diagnosis of familial- or infectious-associated hemophagocytic lymphohistiocytosis and who required intensive care support for organ failure. INTERVENTION: None. MAIN RESULTS: During a 10-yr period, 5,439 children were hospitalized in our PICU. A total of 11 children were diagnosed as suffering with HLS. Of these 11 patients, three (27%) had the familial form and eight had the infectious-associated form. After admission to the PICU, seven patients (63%) were diagnosed as suffering with HLS and each had one or more organ failures (patients 3-7, 9, and 10). All presented with fever, hepatomegaly, and splenomegaly; in addition, all had at least two of the following: anemia, neutropenia, or thrombocytopenia. All 11 had lymphohistiocytic accumulation in bone marrow (n = 10), lymph node (n = 2), lung (n = 2), and/or liver (n = 1). Organ failure was noted most often in the respiratory system (n = 7) attributable to severe, acute respiratory distress syndrome and pleural effusion. Of the 11 patients, six had cardiovascular involvement that manifested as shock in three and as capillary leak syndrome in three. Renal failure occurred in four patients. Of these, two required hemodiafiltration and one required peritoneal dialysis. Liver failure occurred in three and central nervous system involvement and coma in three. Most of the patients required massive therapeutic intervention, including assisted ventilation (n = 6), inotropic support (n = 3), and hemofiltration (n = 3). A total of seven patients (63%) died. CONCLUSIONS: Hemophagocytic lymphohistiocytic syndrome in the pediatric population may have a dramatic clinical picture, with multiple organ failure as a presenting symptom or early in the disease course, mandating intensive support in the PICU.

[From IgE to the cell adhesion molecule]. / De l'IgE à la molécule d'adhésion.

The aim of this work is to contribute to increasing our knowledge of asthma. The IgE can't alone be imputed in the anaphylactic reaction. Asthma is a bronchial inflammatory disease. The secretion of lymphokines increases the inflammatory response. The eosinophils cells damage the respiratory epithelium. The mast cells and the basophils cells release the chemical mediators and also the cytokines. The adhesion molecules situated in the cell membrane permit leukocyte cells to integrate with extracellular matrix during intra-tissue migration. Adhesion molecules may play a primary role in the pathogenesis of inflammatory response. The key role of the CD4 + T lymphocytes subset appeared in the last years.

[Acute paraffin oil pneumopathies. Apropos of a case in a fire-eater]. / Les pneumopathies pétroliques aiguës. A propos d'un cas chez un cracheur de feu.

A case of acute paraffin oil-induced pneumonia due to accidental inhalation by a fire-eater of kerdane, a petroleum derivative is reported. The symptoms and course of respiratory manifestations of acute paraffin oil poisoning are reviewed. The physical properties of the petroleum derivative inhaled account for the pathogenesis of the pneumonia. Pulmonary lesions, usually fully reversible, result from the joint effects of an inflammatory phase with exudate and a proliferative phase.

[Poisoning by diazomethane inhalation]. / Intoxication par inhalation de diazométhane.

A case of Diazomethane intoxication has been reported. Diazomethane is gaz commonly used by pharmacological and industrial chemist. This case of intoxication is rare, since only 15 other cases have been published up to now. A review of the literature leads leads describe the clinical feature of such an intoxication by gaz inhalation. When Diazomethane is inhaled it induces respiratory and general disorders. In some cases, it be fatal, and, in some other cases, when a second exposure happens, bronchial asthma may appear. Post mortem studies show pulmonary oedema lesions with important inflammatory reaction localized in the peribronchia. It must be emphasized that direct aggression is probably responsive of toxical discorders, but increase of the symptoms consecutive to a second exposure are related to allergical mechanisms.

[Possible role of committees concerned with the fight against tuberculosis and respiratory diseases in home ventilatory assistance of severe chronic respiratory insufficient patients (author's transl)]. / Role possible des comités de lutte contre la tuberculose et les maladies respiratoires dans l'assistance aux insuffisants respiratoires chroniques graves par la ventilation à domicile.

It appeared that the departmental committees involved in the fight against tuberculosis and respiratory diseases were qualified to bring assistance to severe chronic respiratory insufficient (C.R.I.) patients. This modified course of action replaces their previous struggle against tuberculosis due to its recession. In Seine-Maritime the committee has signed an agreement with the principal Social Security organizations. This agreement defines the modalities and cost of home care for severe C.R.I. patients who have been tracheostomized permanently and whose condition justifies the continuation of assisted ventilation. Contingent on certain medical and socio-family conditions, the return home of such patients is possible. Thus in 15 years we have been able to benefit 72 patients with assisted endo-tracheal ventilation at home. Thirty-two of them are actually still living.

[Respiratory function disorders during hepatopathies with immunity involvement]. / Perturbations de la fonction respiratoire au cours des hépatopathies a participation immunitaire.

A respiratory functional test was done systematically on 24 patients with chronic hepatitis and was found normal in only 2 cases. It showed an alteration of the transfer in 14 patients and a syndrom of hyperinflation in 8 others. Despite the histopathological proof it seems that the transfer alteration could be attributed to diffuse interstitial pulmonary lesions. They have already been noted in chronic hepatitis by several authors. The pathogeny of this interstitial involvement probably implied immunity phenomenons including the participation of the antigen Australia or others. As for the hyperinflation syndrome observed in 4 patients in the absence of any bronchopulmonary history, it could reveal an emphysema, which, so far, had not been seen associated to chronic hepatitis.

[Study of P50 and 2,3-diphosphoglycerate in 23 bronchoemphysema patients as a function of hypoxia and hemoglobin concentration]. / Etude de P50 et 2,3-diphosphoglycérate chez 23 broncho-emphysémateux en fonction de l'hypoxie et de la concentration d'hémoglobine.

2,3-DPG and P50 were measured in 23 patients with chronic obstructive lung disease. All patients had a chronic hypercapnia (PaCO2 greater than or equal to 45 Torr). They are shared in 3 groups, according to hemoglobin content [Hb] and hypoxia : group I(PaO2 = 48.0 +/- 2.4 Torr ; [Hb] = 15.9 +/- 0.3 g. 100 ml-1; n = 9), (M +/- 1 SE); group II (PaO2 = 46.4 +/- 5.0 Torr; [Hb] = 11.6 +/- 0.7 g. 100 m[-1; n = 7); group III(PaO2 = 61.4 +/- 2.4 Torr; [Hb] = 13.3 +/- 0.4 g. 100 ml-1; n = 7). 2,3-DPG (group I : 1.05 +/- 0.06 mole.moleHb-1; group II : 1.02 +/- 0.08; group III : 1.11 +/- 0.08) was not significantly different of 2,3-DPG value of 12 control subjects (0.96 +/- 0.04). P50 of group I (26.9 +/- 0.9 Torr) and group III patients (28.1 +/- 1.6 Torr) was not significantly different of control value of P50 (27.4 +/- 0.5 Torr). P50 of group II patients (29.6 +/- 0.8 Torr) was significantly higher than P50 control and group I values (p less than 0.05). All the patients of group II died. These results suggest that in patients with chronic obstructive lung disease : 1) P50 value is different with various clinical conditions; 2) P50 increase is a compensatory mechanism in severe hypoxemia with anemia, but is not sufficient; 3) [Hb] is the best data for clinical prognosis.

[Respiratory function tests in epidermoid cancers of the bronchi]. / A propos de l'exploration fonctionnelle respiratoire au cours du cancer épidermoïde des bronches

The results of functional tests are analyzed in 50 cases of epidermoid bronchial cancers. Severe disorders take place in almost every patient. In one patient out of two, there is an old distension already existing prior to the cancer; in other patients gas exchange disorders can be improved by the intervention. Moreover, from some results a spreading of cancer towards the mediastinum is to be feared.

[Information obtained by liver biopsy in 100 tuberculous patients]. / Renseignements fournis par la biopsie hépatique chez 100 malades tuberculeux

The results of liver biopsy in 100 patients with tuberculosis are reported. In 8 patients, biopsy only occurred secondarily, during liver disease which appeared during antituberculous treatment. In five cases, the association of rifamycin and isoniazid was probably responsible and the mild histological signs noted suggested a favourable course after stopping one of the drugs or simply reducing the dose. The 3 other patients had virus hepatitis and biopsy was of prognostic interest by revealing the onset of post-hepatic cirrhosis. In 92 cases, liver biopsy was carried out before treatment. In 34 cases the liver was normal, in 38 patients there were hisotlogical changes which did not suggest tuberculosis but, probably, alcoholism. These were : steatosis, in 21 cases, cirrhosis in 8 cases, a mixture of steatosis and cirrhosis in 4 cases, and acute alcoholic hepatitis in 5 cases. Finally, in 20 cases, biopsy revealed an appearance of granulomatous hepatitis. Although this lesion is significant in the development of the disease, it is not characteristic of tuberculosis unless there is caseous necrosis, as in 2 cases, and unless culture of the biopsy material is positive, as in one case out of 9, i.e. the diagnostic interest of liver biopsy is not very great compared with prognostic interest. By determining the anatomical condition of the liver, often not obvious when simple liver function tests are carried out, it permits one to forsee to some extent the tolerance of the liver to antituberculous treatment, especially in alcoholics.