BACKGROUND:N-terminal brain natriuretic peptide (NT-proBNP) is a neurohormonal substance secreted mainly by the cardiac myocytes of the left ventricle and to a less degree of the left atrium. The releasing mechanism is induced by an increased wall stress on the base of volume or pressure load of the ventricle. The physiologic actions of BNP are prohibition of the sympathetic activity, vasodilatation, natriuresis, diureses and inhibition of the renin-angiotensin system. Because of its high sensitivity and specifity in relation to the left ventricular incompetence, BNP as well as NTproBNP are well accepted markers of ventricular dysfunction. It was the aim of the study to establish reference values of NT-proBNP serum concentrations throughout childhood, in particular in the newborn age group.METHODS:In a cross sectional study, serum NT-proBNP concentrations were measured by an ElectroChemiLuminescenceImmunoAssay ("ECLIA" Roche) in the umbilical cord blood of 62 healthy full-term neonates and in 222 healthy probands from birth up to the age of 18 years.RESULTS:The concentration of NT-proBNP in the cord blood samples ranged from 281 to 2595 pg/ml (mean: 818 pg/ml). There was a significant increase in the first days of life followed by a rapid decrease during the first year and a further gradual decrease throughout infancy; beyond the 10(th) year of age normal adult values were approached.CONCLUSIONS:The knowledge of the normal range of plasma NT-proBNP levels in healthy subjects during childhood serves as a non-invasive marker for differentiation of pathologic conditions such as acute myocarditis, hypertrophic or dilated cardiomyopathy, tachyarrhythmias and pulmonary hypertension.

Laser induced fluorescence of Na atoms excited inside the evanescent wave of a glass prism is spectrally resolved for the first time, showing a pronounced dependence of linewidth on the surface temperature in the temperature range of 293 K to 435 K. We are able to reproduce the measured fluorescence lines quantitatively by use of the perturbative density matrix theory. The good agreement between theory and experiment emphasizes the fact that the response of the gas to the evanescent field arises mainly from the atoms desorbing from the surface and is of nonlocal nature.

This report is upon a case with fetal hydrops on the base of a paracardiac cystic lymphangioma in the mediastinum. The newborn which had hypoplastic lungs and multiple pneumatothoraces died because of a pulmonary insufficiency. An overview is given about the most common causes of the nonimmune fetal hydrops, and the pathomechanisms are discussed.

This report is upon a case with fetal hydrops on the base of a paracardiac cystic lymphangioma in the mediastinum. The newborn which had hypoplastic lungs and multiple pneumatothoraces died because of a pulmonary insufficiency. An overview is given about the most common causes of the nonimmune fetal hydrops, and the pathomechanisms are discussed.

We report six patients with Emery-Dreifuss muscular dystrophy (EDMD) and four patients including one female with EDMD phenotype (EDMDP). This series includes one sporadic case who had previously been reported in this journal under the diagnosis of "rigid spine syndrome" in 1977. Time of observation ranged from three to ten years. Detailed cardiological assessment was performed in all patients, skeletal muscle biopsies were obtained from 9 out of 10 and cardiac muscle biopsies from 2 out of 10 patients. One patient showed evidence of cardiomyopathy in the absence of clinically apparent neuromuscular disease and one sibling of another EDMD patient reportedly had a similar combination of symptoms which, to our knowledge, has not yet been reported. Cardiac involvement was found to consist of four independent, albeit often combined features: 1) impairment of impulse generating cells; 2) conduction defects with atrial preponderance; 3) increased atrial and ventricular heterotopia; and 4) functional impairment of ventricular myocardium. Ventricular involvement as apparent from ventricular heterotopia, abnormal enddiastolic diameter, decrease of contractility and/or morphological evidence of ventricular myocardial disease was found in 7 out of 10 patients and confirmed by myocardial histopathology in two EDMD patients. In one myocardial biopsy extensive accumulations of intermediate filaments were observed, a rare finding, which has not been linked to EDMD before. Skeletal muscle biopsies showed evidence of myopathy throughout but several equivocal features such as fibre type grouping in EDMD and fibre type disproportion in EDMDP were also observed. The variability of clinical manifestation of both cardiac and neuromuscular disease encompassed a broader spectrum than apparent from the literature. The consequences for the inherent differential diagnosis are discussed.

On the basis of a case of multiple, extra-adrenal intrathoracic pheochromocytomas (total of 6 tumors), the value of dynamic computerized tomography investigations, venous sampling, scintigrams with 131I-meta iodobenzylguanide, and selective arteriography is discussed.

: It is reported on a nine year old boy with the oligosymptomatic form of the septo-optical dysplasia de Morsier: congenital nystagmus with nearly complete loss of visus due to a hypoplasia of the opticus-nerve of both sides, growth retardation (dwarfism) because of STH-deficiency and lack of the gonadotropins. The septum pellucidum is present. The cause of this embryologic malformation within the prosencephalon is not known.