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There is an almost 40-fold difference in incidence rates of symptomatic coeliac disease between Denmark and Sweden. In an attempt to explain this difference, the present study focused on the interobserver agreement when pathologists were assessing small intestinal biopsy specimens from children suspected of suffering from coeliac disease. The study was performed on 90 biopsy specimens from 73 children. Most of the biopsies came from children who turned out not to suffer from coeliac disease after a clinical evaluation including small intestinal biopsy. Using the kappa methodology, the interobserver agreement between two Danish pathologists and one Swedish pathologist, all of whom were experienced, was "moderate" to "substantial" or 0.57-0.75. Kappa indices when the pathologists evaluated selected histological elements were in the interval from 0.24 to 0.67. A comparison of a previous routine diagnostic assessment of the 90 biopsies (14 pathologists) with the results of the experienced pathologists in the present study gave kappa indices of from 0.53 to 0.57. The study could prove no major differences in the histopathological assessment of small intestinal biopsy specimens made by Danish and Swedish pathologists. The difference in clinical presentation of coeliac disease in Denmark and Sweden does not relate to differences in the histopathological assessment of small intestinal biopsies.

In order to elucidate the role and aetiology of chorioamnionitis in stillbirth a case referent study was carried out in 58 pregnant women with late foetal death (cases) and in 58 pregnant women at term with live foetus (referents) matched for age and parity in Maputo Mozambique. Samples from women, stillborns and liveborns, were collected for microbiological and histological assessment. Histological chorioamnionitis was diagnosed in 96% of the cases and in 67% of the referents (OR = 13.5; 95% CI: 2.9-123.9). Escherichia coli was the species most frequently isolated in stillborns; in 14/16 (88%) cases it was isolated from intracardiac fluid. E. coli was associated with chorioamnionitis in 28% of the stillborns as compared to 5% of the referents (OR = 6.9; 95% CI: 1.4-65.4). No group B streptococci were recovered from any placenta or newborn. Vasculitis was present in 12 (21%) cases and in 3 (5%) referents (OR = 4.8; 95%, CI: 1.2-27.7). Histological chorioamnionitis was thus associated with stillbirth. E. coli was common in stillborns. The presence of vasculitis in one fifth of the stillborns indicated that the foetus was alive at the onset of infection.

Two cases of parietal cell carcinoma are reported and compared with seven cases previously described. Eight of nine patients have been male. The tumours grow mainly outside the mucosa and can macro- and microscopically be misinterpreted as leiomyoblastomas or lymphomas. The survival, also with metastases, is long. The proper diagnosis requires ultrastructural investigation.

The lysosomal enzyme beta-hexosaminidase (Hex) was studied immunohistochemically with a monoclonal antibody in normal human livers and in livers with cirrhosis and cholestasis. No reaction was detected in sections from normal livers. The reaction in diseased livers was prominent mainly in degenerating hepatocytes, whereas Kupffer cells and bile duct epithelium showed faint reactivity. The reaction product was intense in degenerated hepatocytes, especially in the periphery of the pseudolobules in cirrhotic livers. In cholestatic livers the positivity was localized around central veins in cells with bile pigment. The more pigment, the stronger was the reaction. It is suggested that the elevated level of serum Hex found in cirrhosis and cholestasis derives from degenerating hepatocytes and activated Kupffer cells.

56 human liver biopsy specimens with insignificant or no histological changes, but with abnormally strong canalicular alkaline phosphatase activity, were studied histochemically for other enzyme changes. In comparison with normal specimens, more extensive and increased canalicular activity of gamma-glutamyl transferase, and increase of canalicular leucine aminopeptidase, was found, while the sinusoidal activity of the latter enzyme was decreased. Staining for adenosine triphosphatase regularly desclosed the normal pattern of sinusoidal and canalicular activity. The lysosomal enzymes, acid phosphatase and beta-glucuronidase, stained more intensely than ordinarily, while the reactions for enzymes present in the cytosol (lactic dehydrogenase), in the mitochondria (succinic dehydrogenase, imonoamine oxidase) and in the endoplasmic reticulum (glucose-6-phosphatase) were normal.

A child with multiple anomalies, including growth retardation, a left-sided diaphragmatic hernia with lung hypoplasia, and cerebral malformations is described. Cytogenetic investigation demonstrated a deletion of the distal part of one chromosome 15, del(15)(q24qter), an aberration not previously described. Family studies revealed that the mother had a balanced translocation, t(6;15)(p25;q24). Two of her subsequent pregnancies resulted in abortions after prenatal diagnosis: one fetus was trisomic for 15q24----qter, while the other had monosomy 15q24----qter and a left-sided diaphragmatic hernia similar to the first child.

Histochemical demonstration of the alkaline phosphatase activity with an azo-dye method in human livers showed that the activity, which is normally localized to endothelial cells of portal and central veins as well as of sinusoids around the central veins and to a lesser extent of sinusoids around the portal connective tissue, is increased in various liver diseases. Appreciable canalicular activity is rare, and when it occurs it is more prominent in parenchyma involved by malignant tumours.

The alkaline phosphatase reaction is normally absent in human bile canaliculi, but was found in 79 patients. In search for a common causal factor, these patients were further examined. Thirty-seven were autopsied. The conditions most ocmmonly associated with the phenomenon were malignant tumours with or without involvement of the liver, collagen diseases, long-standing partial obstruction of the common bile duct, and genetic variants of alpha-1-antitrypsin. No clinical or laboratory facts were common to all the patients.

To evaluate the frequency of false positive globular inclusions, 89 autopsy cases with various malignancies and liver metastases have been examined by immunoperoxidase staining of liver sections and isoelectric focusing of sera. Four subjects with AAT globules in their hepatocytes were found, all of whom had the Pi Z phenotype. Globular inclusions were not found in any subject lacking the Pi Z allele on isoelectric focusing, but in 3 subjects with the Pi Z phenotype no hepatocytic globules were found.

A 17-month-old girl with a vaginal embryonal carcinoma (germ cell tumour) is reported. Alpha-fetoprtoein (AFP) was elevated before treatment in blood and vaginal fluid. Normal levels of AFP in blood and vaginal fluid were found after radiation and comination chemotherapy. By an indirect immunoperoxidase staining AFP was demonstrated in tumour tissue. 24-month-old local recurrence was preceded by a slight increase in serum AFP, and AFP could also be demonstrated in vaginal fluid and vaginal smears.

The histochemical patterns of the alkaline phosphatase reaction in liver specimens obtained at 185 consecutive autopsies were studied for any correlation with clinical data and post mortem findings. Alkaline phosphatase activity of bile canaliculi was found in 71 per cent of the subjects with malignant tumours not involving the liver, and in 77 per cent of the subjects with malignant tumours involving the liver. The histochemical pattern did not differ with the type of tumour. Most subjects with rheumatoid arthritis as well as most of those with centrolobular hepatic necrosis due to heart incompensation also showed alkaline phosphatase activity in the bile canaliculi.

The histochemical enzyme pattern in normal, extra-hepatic bile ducts from rabbits was studied. A difference between the duct epithelium and the crypt epithelium was noted mainly in the activity of gamma-glutamyl-transpeptidase and alkaline phosphatase which only stained positively in the crypts. No difference from the normal enzyme pattern was noted after diversion of the bile flow for up to 30 days.

Two unrelated families are presented with repeated occurrences of a congenital syndrome of which the main stigmata were polycystic kidneys and occipital encephalocele (Meckel syndrome). Prenatal diagnosis, followed by interruption of pregnancy, was performed in one case. The diagnosis was based on an increase of amniotic alpha-fetoprotein (AFP), and on the mode of growth and cell types of cultured amniotic cells. In another similarly examined case the diagnosis was suspected, but the parents did not wish the pregnancy to be interrupted. The child was stillborn and malformed. AFP values are presented and discussed in relation to the observed malformations. Neural tube defects are associated with an increase of AFP in amniotic fluid, but, as in normal pregnancies, the values decrease with increasing gestational age. On the other hand, kidney malformations seem to be associated with AFP values which remain high or even increase with increasing gestational age.