In 1930 adenosine triphosphate appeared in the literature from W. A. Engelhardt's work on avian erythrocytes. This was an early example of oxidative phosphorylation in intact cells, and it required methylene blue and oxygen. Both Belitser and I realized that the use of Warburg manometers for aeration was critical in order to generate oxidative phosphorylation of glucose in tissue preparations. Test tube techniques did not work. In 1956 we were able to describe a human type of diabetes called "galactose diabetes," in which consumption of human or cows' milk provokes mental retardation. Replacement of human or cows' milk products with "vegetable milk" formula in early infancy can prevent retardation. We determined that the disease results from a defect of galactose-one-phosphate uridylyl-transferase, a hereditary enzyme. This type of enzyme defect, if discovered and treated in early infancy, is a benign molecular disease. Regulation of transport systems in mammalian cell cultures are frequently complex energized systems. Perhaps my greatest surprise in this regard was the mere fact that an all-cis "odd" hexose-D-allose turned out to be a highly intense down-regulator of the hexose transport system. Additions of inhibitors of oxidative phosphorylation (such as oligomycin or di-nitrophenol) arrested the allose-mediated down-regulation. We have reason to suspect that the strong down-regulator is a phosphorylated form of D-allose. Thus ends my story about oxidative energized biological phosphorylation systems.
The first children's hospital in Sweden (Kronprinsessan Lovisa's Children's Hospital) was established in Stockholm in 1854. In 1885 it was divided into a medical and a surgical department. This constituted the birth of pediatric surgery in Sweden. Pediatric surgery has been included in undergraduate teaching programs since 1945. A personal Associate Professorate in Pediatric Urology was instituted at the Karolinska Medical School in Stockholm for N. O. Ericsson in the late fifties. Upon his retirement in 1976 this personal chair was converted into an established Professorship in Pediatric Surgery. Pediatric surgery has been recognized as a specialty by our Medical Association since 1947. A survey of the Annual Reports from the Lovisa Hospital from 1885 to 1969 shows three phases in the development of our specialty in Sweden. The first stage extends from 1885 to 1932. During this period the disease pattern was dominated by septic and tuberculous infections, by empyemas, and by ENT diseases. Few cases of congenital malformations were reported. The bulk of general surgery in childhood was performed in the departments of general surgery. The second stage (1932-1945) was characterized by a decreasing incidence of tuberculous infections, by a successive transfer of orthopedic and ENT patients to the Departments of Orthopedic and ENT surgery respectively. During this period, a marked increase occurred in the volume of malformation surgery. This was due to the centralized treatment of congenital anomalies. The third stage started in 1945. The war had ended and we became acquainted with the dramatic development of pediatric surgery in other countries, in particular in the USA. The main advance was the possibility of opening the chest for repair of congenital anomalies of the heart and the great vessels, of the esophagus, and of the diaphragm. Soon afterwards, rectosigmoidectomy was introduced for the treatment of Hirschsprung's disease. In 1952, a second department of pediatric surgery was opened in Stockholm as a part of a new Children's Clinic at the Karolinska University Hospital. The major part of general surgery from the Stockholm area and all of the cardiovascular surgery stayed with the "Lovisa Hospital", while the major part of neonatal surgery and specific abdominal surgery was performed at the Karolinska Hospital. In order to improve the rather underdeveloped situation of pediatric urology, N. O. Ericsson was appointed to the post of Associate Head of the Karolinska Department, soon bringing this field to the frontlines of international standards. This historical review ends with some of the author's personal memories from the last 50 years.
Between 1888 and 1892, Mikulicz as well as Fuchs observed each a case of oculo-salivary glandular syndrome. Ten years later, Heerfordt described uveitis complicated by swelling of the lacrimal and salivary glands. Within 100 years, the interpretation of this disease changed repeatedly and considerably: infection of particularly exposed organs--non-avirulent tuberculosis--salivotropic virus--Boeck's disease--allergic-hyperergic reaction--diencephalic and nervous dystrophy with segmental projection--(auto)immune disease--oculo-salivary complex including Sjøgren's syndrome--all these were discussed as possible aetiologies. Short biographies of Johannes von Mikulicz-Radecki, surgeon at Austrian and Prussian universities; Ernst Fuchs, ophthalmologist of Vienna; Christian Frederik Heerfordt, a Danish ophthalmologist particularly fond of publicity.
The combination of neonatal intrahepatic cholestasis and lymphoedema in feet and legs is a specific syndrome named after the Norwegian paediatrician Øystein Aagenaes, who described the syndrome in 1968. The condition is autosomal recessively inherited and the gene is located to 15q, but not yet identified. The condition is particularly frequent in the southern most part of Norway and the gene frequency is estimated to be about 3%. The development of small lymphoid vessels is probably deficient around the small biliary tracts and in general. Aagenaes' syndrome is found in patients from other parts of Europe and the US, but more than half of the cases are of Norwegian origin.