Rectal carcinoids as tumors of the hindgut endocrine cells: a morphological and immunohistochemical analysis.

The rectal mucosa is richly endowed with a constellation of amine and polypeptide hormone-producing endocrine cell types which may be identified by silver staining and immunohistochemical methods. In order to study the relationships of rectal carcinoid tumors to the normal hindgut endocrine cells, rectal carcinoids and normal rectal mucosa were compared for the presence of argentaffinity and argyrophilia and for the distribution of a battery of polypeptide hormones. Normal rectal mucosa contained frequent cells which stained for bovine pancreatic polypeptide (PP), human PP, and glucagon-like immunoreactivity (GLI. Somatostatin (SRIF) was present in a smaller proportion of rectal endocrine cells. Both argentaffin and argyrophil cells were encountered frequently in normal rectal mucosa. In the series of 13 rectal carcinoids examined, two cases were focally argentaffin-positive, while eight tumors revealed varying degrees of argyrophilia. Eight tumors contained immunoreactive bovine PP, and four of these tumors which were tested for human PP were also positively stained. SRIF was present in five cases, while GLI was identified in two tumors. Four of the tumors were multihormonal. Rectal carcinoids have a rich polypeptide hormone content which parallels that of the normal rectal mucosa. The distinctive hormonal profile and silver staining properties may prove to be of value as specific markers for carcinoid tumors of rectal or hindgut origin.

Sequential serum aluminum and urine aluminum: creatinine ratio and tissue aluminum loading in infants with fractures/rickets.

Aluminum toxicity is associated with the development of bone disorders, including fractures, osteopenia, and osteomalacia. Fifty-one infants with a mean (+/- SEM) birth weight of 1007 +/- 34 g, gestational age of 28.5 +/- 0.3 weeks, and serial radiographic documentation at 3, 6, 9, and 12 months for the presence (n = 16) or absence (n = 35) of fractures and/or rickets were studied at the same intervals to determine the serial changes in serum aluminum concentrations and urine aluminum-creatinine ratios. Autopsy bone samples were used to determine the presence of tissue aluminum. Serum aluminum concentrations from 46 infants were stable and similar between groups, with mean values between 15 and 22 micrograms/L. Urine aluminum-creatinine (micrograms per milligram) ratios from 14 infants were higher in infants with fractures and/or rickets (0.26 +/- 0.06 vs 0.12 +/- 0.04) at onset, and rate of decrease in aluminum-creatinine ratio was faster in infants without fractures and/or rickets. All but three infants were tolerating complete enteral feeding at all sampling points. One infant who received aluminum-containing antacid had marked increase in serum aluminum to 83 micrograms/L while urine aluminum-creatinine ratio increased from 0.09 to a peak of 8.53. Vertebrae from three infants at autopsy (full enteral feeding was tolerated for 37 and 41 days in two infants, respectively) showed aluminum deposition in the zone of provisional calcification and along the newly formed trabecula.(ABSTRACT TRUNCATED AT 250 WORDS)

Two fetal deaths associated with maternal sepsis and with thrombosis of the intervillous space of the placenta.

The placental pathology in two second trimester fetal losses associated with mild maternal disseminated intravascular coagulation are reported. Case one had a dental abscess, a leukocytosis of 36300 white blood cells/m, and evidence of mild consumptive coagulopathy at 20 weeks. Case two had septic findings including disseminated intravascular thrombosis associated with pyelonephritis. The placentae had extensive intervillous thrombosis at the periphery of spiral arterial flow. It is hypothesized that in mild disseminated intravascular coagulation, the trophoblast inhibits fibrinolysis, favouring thrombosis perhaps due to production of plasminogen activator inhibitor.

Chronic deciduitis in the placental basal plate: definition and interobserver reliability.

This study tested whether concordance could be achieved for abnormal inflammation in the basal decidua of placental specimens among 6 pathologists experienced in placental pathology. Thirty microscope slides were evaluated by the pathologists for chronic deciduitis. They also scored the severity and extent of inflammation and the presence of plasma cells. No definition of chronic deciduitis was provided. Concordance (5/6 or 6/6 agreement) was achieved in 23 cases (76%). Spearman's rank correlation showed that the diagnosis of chronic deciduitis was almost identical to the assessment of the severity of the inflammation. A regression analysis showed that the perception of severity (and hence chronic deciduitis) was influenced by the other 2 variables, extent and plasma cells. The results were shared with the pathologists, and 25 cases (excluding those with previous 6/6 consensus) were reevaluated. Concordance was now achieved in the 83% of those remaining cases. Using a threshold based on the severity and the extent of lymphocytes, and the presence of plasma cells, pathologists are able to diagnose chronic deciduitis with sufficient concordance to be of value in clinical correlation studies.

Observer reliability in assessing placental maturity by histology.

AIMS: To evaluate the ability of five experienced perinatal pathologists to assess placental maturity reliably by histology. METHODS: Twenty four haematoxylin and eosin slides, six each from placentas of 27, 31, 35, and 39 weeks' gestation, were circulated to five pathologists on three separate occasions. The slides were labelled with the correct or incorrect gestational ages. RESULTS: The mean absolute error over all 360 readings was 2.72 weeks. Only 54% of the slides were assessed within two weeks of the correct gestation. Pathologist tended to overestimate younger gestations and underestimate older gestations. Two, and possibly three, pathologist were influenced by the gestational age state on the label. One pathologist, who did not appear to be influenced by the label, was more accurate in diagnosing gestation of the placentas than other colleagues. CONCLUSIONS: Experienced pathologists can have difficulty in assessing the villous maturity of placentas by histology. They can also be influenced by clinical information provided, such as gestational age. Other observer reliability studies must address the issue of the influence of labelled information on observer variation. A difference in maturation would have to be of a six week magnitude to have a chance of being detected by current methods. This may limit the value of the histological diagnosis of placental dysmaturity as a surrogate marker for uteroplacental ischaemia.

Umbilical artery occlusion and fetoplacental thromboembolism.

BACKGROUND: To our knowledge, fetoplacental thromboembolism has been described only in autopsy specimens. We report the antepartum diagnosis of an umbilical artery occlusion and neonatal diagnosis of an aortic thrombus and placental emboli. CASE: A gravida at 31 weeks' gestation was referred for evaluation of decreased fetal movement and an enlarged fetal bladder. A two-vessel umbilical cord with a collapsed, echogenic third vessel was noted, whereas views of a normal three-vessel cord were available from an examination 5 weeks earlier. A positive oxytocin contraction test prompted delivery. Neonatal color flow Doppler imaging demonstrated an aortic thrombus below the renal arteries and above the bifurcation. Gross and microscopic study of the placenta demonstrated necrosis of the collapsed umbilical artery and numerous placental emboli. The aortic thrombus resolved gradually, and the infant went home on the 39th day of life. CONCLUSION: Umbilical artery occlusion can be diagnosed ultrasonographically and may be a sign of fetoplacental thromboembolism. Assessment of fetal oxygenation status by biophysical profile or contraction stress test may be helpful in the evaluation of umbilical artery occlusion.

Umbilical artery aneurysm: prenatal diagnosis and management.

BACKGROUND: An umbilical artery aneurysm is an extremely rare lesion. The purpose of this report is to describe the prenatal sonographic characteristics of such a lesion and potential obstetric complications. CASE: A 26-year-old woman, gravida 2, para 1, at 30 weeks' gestation was referred for an ultrasound examination because of "an abnormality of the umbilical cord." An ellipsoid cystic lesion was noted in a single umbilical artery. Doppler and color flow Doppler examinations demonstrated nonpulsatile and turbulent blood flow within the lesion, consistent with a diagnosis of umbilical artery aneurysm. The aneurysm increased in size over time with a progressive decrease in amniotic fluid volume. Despite reassuring bi-weekly antenatal testing and planned delivery by 36 weeks' gestation, the fetus died in utero, probably because of acute umbilical venous compression by the aneurysm. Autopsy confirmed the presence of a large calcified aneurysm of a single umbilical artery. Dissection of the aneurysm demonstrated anatomical patency of the entire artery. The umbilical vein was histologically normal, as were sections of the artery. CONCLUSION: Although extremely rare, an umbilical artery aneurysm is a potentially lethal anomaly. We recommend delivery as soon as fetal lung maturity is assured when this diagnosis is made prenatally.

Maternal floor infarction of the placenta: prenatal diagnosis and clinical significance.

OBJECTIVE: To determine whether maternal floor infarction can be diagnosed prenatally. METHODS: We reviewed the charts of 13 patients with maternal floor infarction confirmed histopathologically to determine the frequency of increased placental echogenicity, fetal growth restriction (FGR), and oligohydramnios. Subsequently, we applied these criteria prospectively to diagnose maternal floor infarction in three cases. RESULTS: Twelve of the 13 pregnancies reviewed retrospectively resulted in small for gestational age infants, of which eight were stillbirths. Fetal growth restriction and oligohydramnios were evident on ultrasound in five pregnancies and a placental abnormality was noted in four; two patients exhibited this complete triad of sonographic abnormalities. Three patients were identified prospectively with maternal floor infarction based on sonographic findings and electively delivered live preterm infants. CONCLUSIONS: Maternal floor infarction is a placental condition with profound risk for FGR and stillbirth. Antenatal diagnosis may improve the perinatal outcome with this condition.

Histopathology of fetal membrane rupture: a review of the literature.

Ideally, the histological examination of the fetal membranes should reveal something of the mechanism that ruptured them. However, like any investigation of the crime, there needs to be careful sifting of evidence and confirmation of the validity of inferences. In the case of membrane rupture, the histology must be correlated with the physics of rupture. The usual mental model of the physics of membrane rupture is based on our everyday experiences with the physical world of water balloons, cellophane wrappers, etc. First reviewed are aspects of that intuitive physical model that are important to understanding the histology. Then, the literature is reviewed for histologic correlation with physical and biological observations of membrane rupture.

Response of preterm infants to aluminum in parenteral nutrition.

Twenty-five preterm infants with birth weights (BW) 540 to 2280 g (20 with birth weight less than 1500 g) and gestational ages 24 to 37 weeks, were studied to determine the response to 2 levels of aluminum (Al) loading from currently unavoidable contamination of various components of parenteral nutrition (PN) solution. High Al loading group (H) received solutions with measured Al content of 306 +/- 16 micrograms/liter and low A1 loading group (L) received solutions with 144 +/- 16 micrograms A1/liter. Urine Al:Creatinine (Al:Cr) ratios (micrograms:mg) became elevated and significantly higher in H (1.6 +/- 0.38 vs 0.5 +/- 0.1, p less than 0.05) at the third sampling point (mean 19 days). Serum Al concentrations were highest at onset in both groups and stabilized with study but remained consistently higher than the normal median of 18 micrograms/liter. Calculated urine Al excretion were consistently low and were 34 +/- 6% vs 28 +/- 5% in the H and L groups, respectively. One infant in the L group who died 39 days after termination of above study showed the presence of A1 in bone trabeculae and the presence of excessive unmineralized osteoid along the trabeculae. We conclude that small preterm infants are able to increase urine Al excretion with increased Al load. However urine Al excretion is incomplete with bone deposition of Al and persistently elevated serum Al concentrations.

The anatomic basis of maternal serum screening.

Fetal serum markers, such as alpha fetoprotein (AFP), must traverse one of two very different pathways to reach maternal serum, either from fetus to amnion fluid, membranes and decidua or from fetal to maternal circulation through the placental villi. Alpha fetoprotein usually enters the amnion fluid through body wall defects uncovered by skin or through urine. Placental AFP leakage may be from villous hemorrhage or injury. These observations from anatomic pathology suggest that biochemical markers may exist to identify the source of elevated maternal serum AFP.

The pathologic findings of the fetal membranes in very prolonged amniotic fluid leakage.

We examined the fetal membranes in five patients with prolonged amniotic fluid leakage. Four patients had a clinical history of fluid leakage of at least six weeks' duration, while, in the fifth patient, prolonged leakage was only an inferred diagnosis. Four of the infants died within the first two days of life, while one infant survived. The pathologic findings were varied. Two cases showed, to our knowledge, a previously unreported subchorionic accumulation of squames, which were presumably from cells that were shed into amniotic fluid. One other case showed a subchorionic foreign-body reaction. The two remaining cases showed only necrosis and hemorrhage.

Megakaryocytosis of the liver in a trisomy 21 stillbirth.

This is a case report of a stillborn infant with duodenal atresia and increased megakaryocytes in the liver sinusoids and in pancreatic connective tissue. A postmortem karyotype of skin demonstrated trisomy 21.

Carcinoembryonic antigen, alpha 1-antitrypsin, and alpha-fetoprotein in the pancreas of patients with cystic fibrosis.

The ductular accumulation of "abnormal mucus" is the key histologic feature in cystic fibrosis. This material is periodic acid-Schiff positive and diastase resistant, suggesting that it is glycoprotein in nature. We used the avidin-biotin-peroxidase method to identify this material using antibodies to the serum glycoproteins carcinoembryonic antigen, alpha 1-antitrypsin, and alpha-fetoprotein on paraffin sections of pancreas obtained from a total of 21 patients: 9 with cystic fibrosis, 5 with chronic pancreatitis, and 7 controls. The control patients had normal pancreatic histologic findings, no alpha 1-antitrypsin or alpha-fetoprotein was demonstrated, and only the ductular epithelium reacted weakly for carcinoembryonic antigen. The pancreas in pancreatitis showed fibrosis, acinar atrophy, and ectasia of the ducts that contained only a small amount of periodic acid-Schiff-positive material. This material reacted weakly for carcinoembryonic antigen and alpha 1-antitrypsin. The appearance of the pancreas in cystic fibrosis was similar to that in chronic pancreatitis. However, the ducts contained a greater amount of periodic acid-Schiff-positive material, mostly in the form of globules that reacted strongly for carcinoembryonic antigen and alpha 1-antitrypsin and weakly for alpha-fetoprotein, as did the ductular epithelium. This study shows that the periodic acid-Schiff-positive material in cystic fibrosis contains at least the three serum glycoproteins and that the accumulation may represent a possible defect in cellular synthesis, assembly, or transport of glycoproteins in the ducts.

A maternal death due to thrombotic disease associated with anticardiolipin antibody.

We report a case of fetal death at 30 weeks' gestation followed by the unexpected death of the mother 28 1/2 hours later. At postmortem examination, extensive small-vessel thrombi were found in the maternal organs, including the uterus, kidney, and heart. In retrospect the mother had evidence of chronic pericarditis and myocarditis. Laboratory tests of antemortem serum demonstrated elevated titers of anti-double-stranded DNA and of anticardiolipin antibodies.

Invasive Candida enteritis of the newborn.

Three premature infants (<800 g) showed invasive Candida at the site of their intestinal perforations. This entity is distinct from Candida peritonitis complicating necrotizing enterocolitis and was uniformly fatal. Recognition and aggressive antifungal therapy may improve outcomes.

Further observations of ocular pathology in trisomy 9.

The ocular pathology in a new patient with mosaic trisomy 9 comprised major anomalies and contrasted sharply with the findings in a previous case reported by us. The ocular changes in this case were, in essence, indistinguishable from those encountered in the most severe form of trisomy 13. Similarities to trisomy 18 and 21 were further evidence of the overlap of ocular findings in autosomal trisomies. There is increasing evidence that most, if not all, chromosomes have some role in regulating ocular embryogenesis.