[Mendelson syndrome in childhood]. / Das Mendelson-Syndrom im Kindesalter.

Mendelson's syndrome is characterized by bronchoconstriction, intraalveolary pulmonary oedema and insufficiency of the right heart. We report an unusual case of Mendelson's syndrome in infancy. The morphological findings, combined with the discussion of the pathophysiological stages, are presented.

[Pathology and pathogenesis of propionic acidemia]. / Zur Pathologie und Pathogenese der Propionazidämie.

Described in this paper are pathomorphological alterations in a male newborn with propionic acidemia. Adequate cases from the international literature are evaluated, and the pathogenesis is discussed. The pathological findings are predominantly degenerative alterations in the brain and proteinaceous deposits in liver, kidney, skin and fatty tissue.

[Pneumococcal infections in children: hemolytic-uremic syndrome]. / Pneumokokkeninfektionen im Kindesalter: Hämolytisch-urämisches Syndrom.

By the example of a case-report diagnostic and therapeutic features of the haemolytic-uraemic syndrome are discussed within the frame of infections by pneumococci. In case of infections by pneumococci but also of those ones caused by other bacteria or viruses neuraminidase may be set free, that on its part may lead to an enzyme-induced haemolysis and in some cases also to a damage of other cell systems. In case of an active share of the kidney a haemolytic-uraemic syndrome may be the consequence.

[Anatomy of the choledocho-pancreatico-duodenal junction in infancy, childhood and adolescence]. / Untersuchungen zur Anatomie der Mündungen von Gallengang und Pankreasausführungsgang im Säuglings-, Kindes- und Jugendalter.

Series of histological sections of the major duodenal papilla were examined. The samples had been taken from human aged between 28 gestational weeks and 19 years. Reflux-preventing structures were found regularly developed in all specimens. Short common terminal canals (duodenal diverticle) were seen in 44 of 46 cases (96 per cent). Pancreatico-biliary union at the level of the duodenal window or below was established even from immature newborns. In two cases, the common bile duct and the main pancreatic duct had orifices in close neighbourhood to each other. They ended on the papilla without formation of a common canal. Abnormal pancreatico-biliary duct union above the duodenal window was not found. Pathophysiological consequences of supra-duodenal duct junction are discussed.

[Morphologic studies of the extrahepatic bile ducts in infancy, childhood and youth]. / Morphologische Untersuchung der extrahepatischen Gallenleiter im Säuglings-, Kindes- und Jugendalter.

Morphology of the extrahepatic bile ducts was examined with light microscopic methods in 55 human cases from the age of 28 gestational weeks up to 19 years. We found that barrel-shaped cells, rod-shaped cells and goblet cells are obligatory components of the bile duct epithelium in infancy. Tunica fibromuscularis of extrahepatic bile ducts consists of connective tissue. Increase of collagen fibers and fibers stained by resorcin fuchsin was observed during development. Neither in hepatic bile duct nor in common bile duct a muscle layer exists. A third of all bile ducts was free of muscles. Sparse muscle bundles in the other cases seem not able to generate bile duct motility. A morphological correlate to the sphincter of Mirizzi and Bernhard we did not find. Tunica adventitia and surrounding connective tissue contain many spacious lymphatic vessels.

[Three dimensional growth of the extrahepatic bile ducts--surgical inferences]. / Körpermassebezogenes Wachstum der extrahepatischen Gallenwege--chirurgische Schlussfolgerungen.

Allometric growth of length and increasing of lumen area of the extrahepatic bile ducts were examined morphometrically in 50 human cases at the age of 28 gestational weeks up to 19 years. We calculated the flow resistance in relation to body weight with the help of the principle of Hagen-Poisoille. Principle ways to better bile flow in cases of non-correctable biliary atresia or bile duct hypoplasia are derived.

[Small intestine biopsies in children with malabsorption syndrome. Autoradiographic studies with 3H-thymidine]. / Dünndarmbiopsien bei Kindern mit Malabsorptionssyndrom. Autoradiographische Untersuchungen mit 3H-Thymidin.

Bioptic material of the small intestine is suitable for short-time in-vitro incubation in 3H-thymidine labelled medium followed by autoradiography. Histological and autoradiographic investigations of the small intestine of 45 children yielded the following characteristic findings: 1. 93 per cent of the healthy controls (n = 14) showed normal mucous membrane (Type I according to Shmerling) and a mean 3H-thymidine labelling index of 13 per cent. 2. Normal histological findings but a mean autoradiographic labelling index of 22 per cent were recorded from 87 per cent of children with cow's milk or fructose incompatibility (n = 12). 3. Mucous membrane of Shmerling Type I and a labelling index of enterocytes of 32 per cent was recorded from 50 per cent of children with coeliac disease (n = 19) after diet periods of seven or three to five months. Other children with coeliac disease but without therapy up to that time showed in 80 per cent the Shmerlings Type III and in 20 per cent Type II accompanied by the highest labelling index of 35 per cent. The increase of proliferative activity of enterocytes in coeliac disease shown by autoradiography and histological observations (villous atrophy) suggested an increased rate of cell death which led to the need for high cell replacement. This autoradiographic method used complementarily to usual histological diagnosis, has proved to help saving an enormous amount of time in the diagnosis of coeliac disease.

[Pathohistologic studies of the upper esophageal blind pouch--a contribution to the discussion of the cause of anastomotic insufficiency following surgically treated esophageal atresia]. / Pathohistologische Untersuchungen am oberen Osophagusblindsack--Ein Beitrag zur Diskussion über die Ursache der Anastomoseninsuffizienz nach operierter Osophagusatresie.

The pathological and anatomical findings obtained in studies of the upper oesophageal blind pouch are described for ten cases of oesophageal atresia where no surgical operations were performed. Figuring prominently in this conjunction are both aplasias and hypoplasias of muscular layers with deficiencies and textural disturbances as well as reductions of intramural ganglia and glands. These particular results are discussed as causes of anastomotic insufficiency after surgical operation for oesophageal atresia. Communications reporting comparable results were not found in literature. From an interpretation of findings it is obvious that the surgical procedure cannot, in many cases, be blamed for anastomotic insufficiency, but that the latter appears to be due essentially to textural disturbances in the oesophageal blind pouches. Also, this would explain why it was not, in the last couple of years, possible to achieve a further reduction in the postoperative lethality rate.

[Pathology of vein, artery and heart catheterization. Studies of autopsies]. / Zur Pathologie des Venen-, Arterien- und Herzkatheterismus. Untersuchungen an Obduktionen.

Among 17,161 autopsies of children and adults 319 cases with complications after catheterization (catheterization of veins, arteries and heart) were analysed. The following pathologo-anatomical findings were established: Haemorrhages, thromboses, thrombophlebitis, incorrect positions of the catheter, embolism after catheterization, perforations of vessels, heart injuries, pleura injuries, pneumo- and haemothorax as well as sepsis and septicopyaemia. The complications were subdivided according to early and late injuries. In 10% the complications after catheterization were directly and indirectly connected with the cause of death. In the clinic the catheterization of the vein is a necessary diagnostic and therapeutic intervention. The evaluation of an extensive autopsy material, however, shows that severe and lethal complications may appear in connection with catheterization. For these reasons a broad use is prohibited. The demand of a strong indication is unrestrictedly to be supported.

[Animal experiment testing of polyurethane soft foam (SYSpur) as an episcleral filling material]. / Tierexperimentelle Testung von Polyurethan-Weichschaum (SYSpur) als episklerales Plombenmaterial.

Soft polyurethane foam was sutured to the sclerae of 28 rabbit eyes in a manner similar to scleral buckling in retinal detachment surgery. During the follow-up period no tissue incompatibility was observed and wound healing appeared to be without complications. Microscopic examination revealed fibrocytes growing through the sponge and after 24 weeks the polyurethane meshwork was completely filled with granulation tissue.

[Occurrence of goblet cells in the human extrahepatic bile duct epithelium in the fetus and newborn infant]. / Uber das Auftreten von Becherzellen im Epithel menschlicher extrahepatischer Gallenwege im Fetal- und Säuglingsalter.

The development of several cell types was studied in human extrahepatic bile ducts of 5 fetuses, 11 newborn infants (gestational age of 28 to 41 weeks), and 3 children younger than 6 years by means of histological and histochemical staining reactions. Barrel-shaped columnar cells and rod-shaped cells in the mucosa are well described in the literature. Goblet cells are only known at the region of the ampulla of Vater. In our study, goblet cells were found in all cases of newborn infants older than 28 gestational weeks. The cytoplasm of the goblet cells contains a mixture of neutral, carboxylated, and sulphated mucopolysaccharides. The physiological importance of the goblet cells is discussed. Parallels to the development and pathology of gall bladder epithelium are elucidated.

[Metabolic triglyceride storage disorders. A report of 2 cases of systemic carnitine deficiency]. / Stoffwechselstörungen mit Triglyzeridspeicherungen Ein Bericht über 2 Fälle von systemischem Karnitinmangel.

Two cases of triglyceride storage in liver, kidney, heart, and skeletal muscle are described in infants who died at the age of 1 1/2 years and 4 d, respectively. In the first patient, a previously normal girl, the clinical symptoms began two months before death with encephalopathy (vomiting, unconsciousness), liver enlargement, hypoglycemia, increase in serum transaminases. These signs disappeared within the following days. Some weeks later she died during the second attack. The 4-d-old boy, the second child of healthy consanguineous parents, showed at the third day of life an impaired sucking, muscular hypotonia, respiratory arrest and bradycardia. An intensive therapy was inefficient. At autopsy gross examination showed only a moderately enlarged yellow liver and an edematous brain in the first case and pale organs in the second one but no cause of death. The microscopial examination of all tissues of both cases showed fat storage within the four organs mentioned above. The common histochemical methods for neutral lipids were positive, the Schultz-reaction for cholesterol and cholesterol esters was negative. The lipid loaden cells did not show birefringence in polarized light. A predominance and strong fat storage of the type I fibres was found in the skeletal muscle. The storage of triglyceride could be confirmed by histochromatography, a thin-layer chromatography of tissue sections. The triglyceride accumulation in liver, heart, kidney, and skeletal muscle is a characteristic feature of systemic carnitine deficiency. The clinical symptoms of the first patient are in agreement with reports of this disease also. A carnitine deficiency in a newborn was not yet described. Family studies revealed a low carnitine concentration in the mother's serum in both cases, while the serum of father and brother resp. sister showed normal carnitine levels.