Schweizerische medizinische Wochenschrift最新文献

Common variable immunodeficiency is the most common symptomatic primary immunodeficiency disease. The patients typically present with a long history of respiratory tract infections, sometimes sarcoid-like lesions and in rare cases boils. Heterogeneity of initial clinical manifestations as well as insufficient knowledge of the syndrome often delay the diagnosis. However, early therapy is important to reduce infections and in particular the development of bronchiectasis. Documenting 19 cases, we discuss initial clinical manifestations, some clinical complications, diagnostic procedures and therapeutic management.

Treatment of the neurogenic voiding disorders which occur after spinal cord injury represents one of the most important challenges of rehabilitation. Inadequate management of neurogenic voiding disorders, especially of urinary incontinence, results in impaired quality of life. Moreover, inadequately treated neurogenic voiding disorders may result in medium and long-term renal complications and even death. Treatment of neurogenic disorders, whatever their origin (spinal cord injury, multiple sclerosis, Parkinson's disease), must take into account the gravity of the neurological disease, the potential risks for the upper urinary tract and the expected quality of life. Therefore, each patient must be considered as a separate entity and treated individually. Recent progress in the comprehension of the neurophysiology of the lower urinary tract and the neurophysiopathology of the neurogenic voiding disorders has been followed by the development of new diagnostic and therapeutic tools aimed at improving the patients' health and quality of life.

Unlabelled: Renal transplantation is the treatment of choice for paediatric patients with end-stage renal failure. Living donor transplantation (LDT) has become an important therapeutic option due to the shortage of cadaver donors and increasingly long waiting times.

Methods: Between 1992 and 1999, a total of 48 paediatric and adolescent patients underwent renal transplantation in Zurich. Of these, 21 patients (44%) received a kidney from a living related donor. 11 patients had been dialysed before LDT over a period of 0.2-5.7 years (median 0.6), and 10 were transplanted preemptively. Triple immunosuppression consisted of cyclosporine A, azathioprine or mycophenolate mofetil (MMF; since 1998), and prednisone. The observation period was 0.5-7.3 years (median 2).

Results: Recipients were 2-18 (median 10.5) years old at transplantation. One third had either a congenital malformation, an inherited disease, or an acquired disorder. One patient died of an associated cardiac disease at 4 months with functioning graft, and one functional graft loss occurred after 2.8 years. 9 patients were switched from cyclosporine to tacrolimus, 7 for biopsy-proven rejection and 2 for cosmetic reasons (hypertrichosis). No antibody preparations were used. Median glomerular filtration rate (51Cr-EDTA), measured after one year in 11 donor/recipients, was 64 (55-95) and 54 (32-82) ml/min/1.73 m2, respectively. The most recent estimated renal function (Schwartz formula) of 19 functioning grafts was 37-79 ml/min/1.73 m2 (median 63). Median body height of 16 patients with no associated extrarenal disease was -0.9 SDS (standard deviation score); the remaining 3--with serious extra-renal disease--were considerably growth retarded. Main complications were reversible rejection episodes in 19 (90%), arterial hypertension (16), CMV disease (2) and asymptomatic CMV infection (3), pyelonephritis (3), and recurrence of the primary renal disease, seizures, diabetes mellitus and non-compliance (one each). Actuarial patient and graft survival (Kaplan-Meier) after 3 years was 95 and 83% respectively. This was not statistically different from the cadaveric donor group (n = 27) with 100 and 80% survival respectively. Overall rehabilitation was excellent. The donors were 12 mothers, 8 fathers and one grandmother aged 31 to 50 (median 39) years; none of them experienced serious postoperative problems.

Conclusions: The paediatric transplantation programme would no longer be feasible in Switzerland without LDT. The results are very encouraging; preemptive transplantation makes it possible to avoid dialysis in half of the patients. The risk for the donor is small, and careful evaluation without putting pressure on the family is essential.

Amiodarone is the most important drug in the treatment of ventricular arrhythmias and is widely used for atrial fibrillation. Thyrotoxicosis, a classical side effect, was thought to be iodine induced, but recent evidence suggests that other mechanisms play an important role (toxic effect on thyreocytes, immunological effects). Thyrotoxicosis due to amiodarone is difficult to treat and is further complicated by the pro-arrhythmic potential of thyrotoxicosis and the fading antiarrhythmic effect after amiodarone withdrawal. The mechanism, diagnosis and therapy of amiodarone-induced thyrotoxicosis are discussed in the light of the available literature.

Giant cell arteritis (GCA) is a systemic vasculitic disease, which in very rare cases causes inflammatory complications such as secondary amyloidosis. We describe a well-documented case, with a clinically mild course, of biopsyproven giant cell arteritis as the only apparent cause of systemic AA-Amyloidosis. The deterioration in renal function due to amyloid deposition occurred rapidly and only a few months after manifestation of giant cell arteritis and was not reversible by steroid treatment. The renal arteries were normal and there was no glomerulonephritis due to giant cell arteritis. This unique case demonstrates that giant cell arteritis with a mild clinical course is closely associated with early-onset severe secondary amyloidosis.

This paper describes some major implications of brain insult following trauma or intracerebral haemorrhage for the development of brain oedema and compromised microcirculation. Secondary insults such as an increase in intracranial pressure and development of contusion and penumbra zone areas, as well as their bearing on outcome, are discussed. A therapeutic protocol is presented which aims at keeping intracranial volume within acceptable limits by counteraction of interstitial brain oedema, reduction in intracerebral blood volume, and improvement of microcirculation around contusions. This ICP-targeted therapy, called the "Lund Concept", for treatment of severe head injury has resulted in marked reduction in mortality following brain trauma.

As medicine becomes more complex and the knowledge base expands, the integration of computer systems into clinical practice would appear to be an inescapable necessity rather than an option. The issues of security and reliability have largely been solved by industrial and business applications of computer technology. The larger challenge lies in designing convenient, efficient and acceptable interfaces between the clinician and computer for data input and presentation. In the future, decision making algorithms are likely to assist the clinician in diagnosis and management to a degree that should significantly improve clinical effectiveness.

Background: The high risks associated with untreated infection in critically ill newborns or children lower the threshold for prescription of antibiotic treatment. Inappropriate use of antibiotic therapy promotes the emergence of resistant strains. This study had three aims: to identify sources of inappropriate antibiotic utilisation, to develop revised guidelines and to implement changes.

Methods: An observational study was performed in a tertiary, multidisciplinary, neonatal and paediatric intensive care unit (PICU) of a university teaching hospital during a 7-month period (456 admissions). Guidelines addressing one of the identified sources of inappropriate utilisation (prophylaxis following surgery) were developed according to published evidence and implemented, and the effect on prescription patterns was assessed during a second observation period.

Results: Patients received systemic antibiotics during 54.6% of all hospitalisation days. Antibiotics prescribed for suspected or proven infection were often continued 1-2 days beyond the intended duration. Prophylaxis accounted for 28% of all systemic antibiotics given, and postsurgical prophylaxis accounted for 14.6% of all exposure days. The literature search revealed little evidence to support this practice. After new guidelines were introduced with the aim of restricting surgical prophylaxis to a single dose prior to surgery, the rate of postsurgical prophylaxis dropped from 14.6 to 11.2% of all exposure days, accompanied by a significant decline in the overall exposure rate from 54.6 to 50.2% of all hospitalisation days.

Conclusion: Several sources of inappropriate antibiotic utilisation were identified. These include failure to discontinue treatment and prolonged prophylaxis after surgery. Implementation of new guidelines reduced antibiotic utilisation.

Cardiopulmonary bypass (CPB) causes a systemic inflammatory response syndrome (SIRS) with activation of neutrophils (increased immature-to-total neutrophil ratio, IT ratio). Does an additional inflammatory response induced by sepsis further increase the IT ratio, so that it can still be used as an indicator of sepsis? In 160 children we analysed retrospectively the IT ratios from the day before CPB to the 10th day after the operation (controls). The 95% confidence limits of the controls were plotted against postoperative day and compared with the IT ratio courses in all children of a 4-year period who developed sepsis during the first 10 days after CPB. All septic children (n = 9) had IT ratios above the upper 95% confidence limits of the controls on the day of positive culture or on the following day. The IT ratio remains a sensitive indicator of sepsis even after CPB.