[Acquired von Willebrand syndrome in two children with congenital heart defects and abnormal haemodynamics]. / Erworbenes von-Willebrand-Syndrom bei Kindern mit hämodynamisch relevantem Vitium: Zwei Fallberichte.

Acquired von Willebrand syndrome is a rare bleeding disorder associated with other primary diseases such as cardiovascular disorders which can cause severe haemorrhage during surgery or interventional procedures. It should be suspected if there is no history of bleeding and abnormal von Willebrand factor (VWF) values in patients with predisposing disorders. We present two children with congenital heart defects and no personal or family history of bleeding. In these patients we preoperative diagnosed acquired von Willebrand syndrome with coagulation tests including analysis of the VWF multimeric pattern.

[Lactobacillus paracasei endocarditis in an 18-yeard-old patient with trisomy 21, atrioventricular septal defect and Eisenmenger complex: therapeutic problems]. / Lactobacillus-paracasei-Endokarditis bei einer 18-jährigen Patientin mit Trisomie 21, atrioventrikulärem Septumdefekt und Eisenmengerreaktion--therapeutische Probleme.

Endocarditis caused by lactobacilli is very rare and so far has been rarely published in adults with cardiac valve diseases especially after dental manipulations. Because of diagnostic and therapeutical problems we hereby report on one case of a female adolescent with Down's syndrome who did not undergo surgical correction of atrioventricular septal defect because of early development of Eisenmenger's syndrome. The onset was subacut and the diagnostic procedures were considerably delayed. Risk factors for the development of endocarditis in this case were preceding antibiotic treatments which increased the risk of selective growth of the causative germs as well as the tricuspidal valve incompetence with simultaneous pulmonary hypertension. The antimicrobial treatment was difficult due to resistance to antibiotic drugs generally applied in such cases and the restricted bacteriological diagnostic methods. Finally we had successfully administered chloramphenicol. The course was complicated by cerebral embolic events. FACIT: Lactobacillus species are facultative pathogenic which should be consideration in cases of subacute endocarditis in children and adolescents with ventricular septal defects and valve diseases. The determination of minimal bactericidal concentration of antibiotic agents and time-kill studies of combined antibiotics are recommended. For initial therapy we recommend high dose penicillin combined with an aminoglycoside. In cases of resistance chloramphenicol should be taken into account as second choice antibiotic drug. The duration of antibiotic therapy should at least over six weeks. In cases of risk systemic embolization is suspected therapy with low dose acetylsalicyclic acid or cardiosurgery should be assumed as therapeutic options.

[Coronary artery fistula--surgical or percutaneous embolization treatment?]. / Koronararterienfistel--chirurgische oder perkutane Embolisationsbehandlung?

Three children aged 4 months, 2.7 and 7 years with the unusual fistula of the left coronary artery to the right atrium were observed over a 2-year period. The two younger children underwent emergency surgery although they showed no clinical symptoms. The reasons for surgical intervention were an aneurysm in the right atrium with obstruction of the vena cava superior and a considerably enlarged fistula, respectively. In the older child, we percutaneously embolized a terminate fistula of the ramus circumflexus with two platinum microcoils without complications. Two-dimensional-echocardiography and color flow mapping were used to confirm the diagnosis. After such diagnosis we recommend a coronary angiography in every case. The transcatheter-coil-embolization is an alternative method to surgical closure in selected cases. We recommend an early onset intervention in case of congenital coronary artery fistula.

Diagnosis of cystic fibrosis by means of particle electrophoresis test.

Incubation of polystyrene latex particles with CF serum leads to reduced electrophoretic mobility (EPM) measured by a particle electrophoresis device. About 85% of 50 CF patients, 60 CF heterozygotes and 190 controls (healthy subjects and patients with different disorders) could be identified correctly.