Kauko Kouvalainen

Main Features of the Congenital Nephrotic Syndrome

Until now 85 patients with an evident congenital nephrotic syndrome have been recorded in Finland. The main features, incidence and etiology of CN are dealt with in this report.

Das kongenitale nephrotische Syndrom

Im Laufe des letzten Vierteljahrhunderts hat man von der klinisch klar umrissenen, aber hinsichtlich ihrer Atiologie noch immer ratselhaften Krankheitseinheit des nephrotischen Syndroms eine bei Neugeborenen und Sauglingen auftretende Nephrose eigenen Geprages zu unterscheiden begonnen: das kongenitale nephrotische Syndrom. Diese Krankheit kommt in Finnland weit haufiger als anderswo vor, weshalb sie dort besonders reges Interesse erweckt hat. In der Kinderklinik der Universitat Helsinki ist versucht worden, die Krankheit von den verschiedensten Seiten her zu erforschen. Die vorliegende Arbeit hat den Zweck, einerseits die vorhandene Literatur zusammenzufassen und andererseits die besonders in Finnland gewonnenen Erfahrungen zu veroffentlichen, was in dieser umfassenden Weise bisher noch nicht geschehen ist. So monoton das Krankheitsbild des kongenitalen nephrotischen Syndroms in Finnland auftritt, so sind in anderen Landern zwar ahnliche, aber doch auch betrachtlich abweichende Falle beschrieben worden, deren atiologische Ubereinstimmung mit der finnischen Krankheit nicht ohne weiteres klar ist. Sofern nicht anders angegeben, sprechen wir im vorliegenden Text vom kongenitalen nephrotischen Syndrom auf Grund der in Finnland gemachten Erfahrungen.

Proteins of Middle Ear Secretion in Serous Otitis an Electrophoretic and Immunoelectrophoretic Study

The protein fractions of the middle ear secretions of 22 patients with true serous otitis were studied electrophoretically and immunoelectrophoretically. The total protein of the secretion varied from 0.4 to 10.5g/100cc, mean 4.5g/100cc. The electrophoretic protein pattern of the secretion was mostly similar to that of the serum. The relative albumin content seemed to be a little lower and that of gamma globulin a little higher than in serum. A very small amount or no γM immunoglobulin was present in the secretion, while γA was clearly demonstrable in little more than half of the cases.

Insulinotropic Activity in the Serum of Obese and Nonobese Infants and Children

ABSTRACT. The ability of a serum fraction, mol wt 1000- 5000, to stimulate insulin release in vitro was studied in 123 obese and normal weight children aged 0-17 yr. The sera were fractionated by serial molecular filtration after treatment with urea. Stimulation of insulin release was determined with a bioassay using isolated rat islets in perifusion. The islet-stimulating activity was found in all obese children less than the age of 10 yr and in the majority of the obese children older than 10 yr of age. In normal weight children the activity was also found in the majority of infants, but was infrequent in older children. The serum islet-stimulating activity was positively correlated with the duration and degree of obesity and with linear growth rate. The molecular structure and origin of the insulinogenic activity in the serum is still unknown. In high-performance liquid chromatography it has the same elution characteristics as the hypothalamic insulin-glucagon liberin. The present results suggest a role for the serum islet-stimulating activity in the pathogenesis of obesity.

50. The Wiskott‐Aldrich's Syndrome — an Immunopathy?

duced. This indicates an immunological similarity between colon antigen (s) and some antigen(s) present in most Enterobacteriace strains. The possible bearing of this findings in the pathogenesis of human ulcerative colitis will be discussed. One might speculate that an infection with a bacterium with antigenic similarities to antigen in colon tissue in an individual with special immunological vulnerability might provoke an autoimmune response which can play a role in the pathogenesis of the disease. We are now engaged in trying to induce experimental colitis in animals and in characterization and purefying the tissue antigen(s) and the cross-reacting bacterial antigen (s ) . DEAE-cellululose and gel filtration through Sephadex G 200. The results indicate that most of the antibodies were macroglobulins ( 19 S) , although antibodies of lower molecular weight ( 7 s) were also present. The antibody titers in sera from ulcerative colitis patients against 8 common E. coli strains did not differ significantly from those found in the controls. However, a significant difference was demonstrated in titers against antigen from E. coli 0 14, known to be rich in an antigen common to most strains of Enterobacteriaee (“Kuninantigen”). By absorbtion of ulcerative colitis sera with antigen from this strain the antibody titer against antigen from germfree ratcolon was significantly re-

Icons in paediatrics: Arvo Henrik Ylppö (1887–1992)

Arvo Ylpp€ o (Fig. 1) was a physician by vocation. He told me that his calling matured in his childhood as he followed his mother’s continuous concern for his numerous siblings and their illnesses. He wanted to become a physician, but not just any physician. His desire was to work as a paediatrician. After matriculation in 1906 – he was top of his class – he enrolled in the University of Helsinki to study medicine and his studies progressed smoothly. At that time, there was a shortage of learning materials in the Department of Anatomy, which slowed down the pace of his studies. A group of devotees, including Arvo Ylpp€ o, decided to take the boat across the Baltic Sea in September 1908 to study anatomy at the University of G€ ottingen. This was a landmark in his future career, as he became more fluent in German and more familiar with the German university system. It also brought him into contact with Professor Friedrich Merkel (1845–1919), who was an anatomist famous for his talent and teaching methods and is remembered for describing the cells that bear his name. Professor Merkel clearly had a profound influence on the future success of the student from Finland who was to become an expert in paediatric pathologic anatomy. Indeed, pathology was to prove a constant factor in Arvo Ylpp€ o’s academic career. When he started the course in paediatrics in Helsinki, Arvo Ylpp€ o spent all of his energy and time learning what was new in medicine. In the summer of 1911, his working life was extremely busy, as he took on the task of what we now call a junior house officer and became a clinical trainee in the Department of Paediatrics. There was a saying about this phenomenal bachelor of medicine in those early years: ‘Ylpp€ o seems to be the paediatrician for all of Helsinki.’ By the time, he approached the end of his clinical training, Arvo Ylpp€ o had repeatedly asked his professor in paediatrics for a topic for a thesis. Strange as it may sound, his professor did not provide this, despite the fact that there was an unfulfilled need for paediatric research. Arvo Ylpp€ o’s reaction was typical of his character and he took his continued education and scientific training into his own hands. Now was the time to return to the German academia that he had learned to know and appreciate since his preclinical studies. In the spring of 1912, he wrote a letter to Professor Leopold Langstein (1878–1933), who was director of the Kaiserin Auguste Victoria Haus, an institution he had founded just a few years earlier under the auspices of the Empress to combat the soaring incidence of early childhood mortality through scientific and clinical efforts. Figure 1 Arvo Ylpp€o at the age of 100, portrayed on a postage stamp published in 1987.

Histologically Demonstrable Cholinesterases and Monoamine Oxidase in th eColon in Hirschsprung's Disease

The occurrence of acetyl cholinesterase ( AChE) and non-specific cholinesterase (ChE) as well as monoamine oxidase (MAO) in the colon in Hirschsprung’s disease was investigated histologically. The investigation comprises a total of 11 cases of intestinal resections. Results: (1) Even ganglia without any ganglion cells contain AChE. ( 2 ) The ChE content appears to be reduced in ganglia in the narrow segment