Multifocal osteomyelitis caused by nontuberculous mycobacteria in patients with a genetic defect of the interferon-gamma receptor.

We describe three patients with multifocal osteomyelitis caused by Mycobacterium avium and a family history of one or more first degree family members diagnosed with various clinical presentations of infections with nontuberculous mycobacteria. There was a significant delay in the diagnosis and they had a protracted course of their illness, which responded only slowly to prolonged multi-drug treatment. In one patient, additional treatment with interferon-gamma (IFN-gamma) was necessary. Macrophages of these patients had decreased in vitro responsiveness to IFN-gamma. Genomic sequencing revealed that these patients and their affected family members were heterozygous for a previously described dominant negative mutation in the gene encoding the IFN-gamma binding receptor-1 chain. The clinical presentations of the infections with nontuberculous mycobacteria in these families, with spread limited to skin, bone and lymph nodes, is discussed in the light of the immune mechanisms that are responsible for the clearance of otherwise poorly pathogenic environmental mycobacteria.

[Hypertension caused by licorice consumption]. / Hypertensie door dropgebruik.

In a 38-year-old woman who was hospitalized because of hypertension and hypokalaemic alkalosis, the intake of liquorice (200 g per day) was proven to be the cause. A liquorice provocation test produced all the expected clinical and biochemical abnormalities. Some kinds of liquorice contain glycyrrhetic acid which inhibits the enzyme 11-beta-hydroxysteroid dehydrogenase (e.g. in the kidney) leading to decreased transformation of cortisol into cortisone. The mineralocorticoid action of cortisol causes a drop in serum potassium and an increase in serum sodium concentration, together with a metabolic alkalosis, which in the patient described led to retention of water resulting in weight increase and hypertension.

Evaluation of an emergency medical system. The prognosis in patients with an out-of-hospital cardiac arrest.

In a retrospective chart review, we evaluated the efficiency of the emergency medical system of the Leiden area in patients with an out-of-hospital cardiac arrest. A total of 309 adult patients were included. Two hundred patients (64.7%) died in the emergency department and 67 (21.7%) died during subsequent hospital stay. Finally, 42 patients (13.6%) survived after hospital discharge. Favourable prognostic factors were the presence of witnesses at the time of arrest, a short call-response interval, an initial cardiac rhythm of ventricular fibrillation or tachycardia, and adequate advanced cardiac life support provided by the emergency medical system. We detected several shortcomings in the system such as an unacceptably prolonged call-response interval for some patients at the periphery of the Leiden area and a delay in the first defibrillation attempt. Improvement of this "pre-hospital chain of survival" is likely to result in a better outcome for these patients.

Prolonged resuscitation efforts for cardiac arrest patients who cannot be resuscitated at the scene: who is likely to benefit?

STUDY OBJECTIVE: To determine who may benefit from prolonged resuscitation efforts after therapy by emergency medical services system (EMS) personnel has failed to restore vital signs. DESIGN: Retrospective chart review. TYPE OF PARTICIPANTS: Two hundred sixteen consecutive adult patients with out-of-hospital cardiac arrest who were admitted to the emergency department without vital signs. METHODS: Identification of prehospital resuscitation data, therapy in the ED, hospital course, and final outcome. RESULTS: Thirty-nine patients (18.1%) were resuscitated successfully. The odds ratio of successful resuscitation in the ED for the patients with ventricular fibrillation at the scene versus those with asystole or electromechanical dissociation was 3.4 (95% confidence interval, 1.5, 7.9). All patients with asystole or electromechanical dissociation, either at the scene or in the ED, died (95% confidence interval, 0, 4.3). CONCLUSION: Prolonged resuscitation efforts in the ED for patients with asystole or electromechanical dissociation usually are futile after previous efforts by the EMS personnel have failed to restore vital signs. Transportation to the hospital may not be indicated. However, for patients with persistent ventricular fibrillation, transport is indicated.

[Pneumococcal pneumonia with a fulminant course]. / Pneumokokken-pneumonie met fulminant beloop.

Four patients admitted with a pneumococcal pneumonia are described. Well-known risk factors such as immunodeficiency or preexisting cardiopulmonary problems were absent. They had been ill for three to five days and had not been treated with antibiotics. Three patients died; upon admission these had extensive pulmonary infiltrates (two or more lobes affected), respiratory insufficiency necessitating mechanical ventilation, and shock at or soon after admission. The prognosis of pneumococcal pneumonia with a fulminant course has not improved in the last few decades despite proper antibiotics and intensive care treatment. Mortality is over fifty percent. Early recognition and treatment can contribute to a better prognosis.

Legionella pneumonia complicated by adult respiratory distress syndrome.

Two patients with sporadic Legionella pneumonia complicated by adult respiratory distress syndrome (ARDS) are described. Details about the clinical course and follow-up of their chest films are provided. The outcome in both patients was favourable. Literature concerning Legionella pneumonia complicated by ARDS is reviewed.

Kawasaki-like syndrome after treatment with mesalazine.

We report a patient who developed a severe hypersensitivity reaction, including rash, lymph node enlargement, fever, hepatitis, and eosinophilia, after sulphasalazine therapy. Five years later, he developed a similar reaction after exposure to mesalazine, the salicylic compound of sulphasalazine. We conclude that patients with known severe systemic reaction to sulphasalazine therapy are also at risk for such a reaction when treated with a 5-ASA preparation.