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Congenital granular cell epulis (CGCE) is a rare tumor of gingiva that is exclusive to newborns, has marked female predominance, and is rarely associated with other abnormalities. Although benign in behavior, CGCE can be lethal by obstruction of respiration and/or deglutition and can require a multidisciplinary team of specialist at birth for survival of an otherwise normal infant. Histologically, CGCE resembles granular cell tumor (GCT), but unlike GCT, which is Schwannian-derived, derivation of CGCE remains an enigma, largely because of its low prevalence. This study presents 24 new cases of CGCE, the largest series since the original description 150 years ago and permits detailed study of homogeneity of cases diagnosed as CGCE as well as detailed comparisons of CGCE with GCT by clinical, morphological, immunohistochemical, and ultrastructural studies. The data show homogeneity within the CGCE cases, more differences than similarities between CGCE and GCT, and no immunohistochemical staining for common placental proteins/hormones in CGCE. The findings support a primitive mesenchymal cell origin, and a progressive degenerative process in CGCE, rather than neoplasia. Prenatal detection of this lesion is important to facilitate adequate preparations for support of these infants during labor and delivery.

Background/objective: Anaerobic exercise capacity is an important component of performing daily activities during childhood. However, diminished anaerobic exercise capacity has been reported in children with chronic conditions. Therefore, the aim of this study was to compare anaerobic exercise capacities between children with familial Mediterranean fever (FMF) and healthy peers., Methods: Twenty-one children with FMF (FMF group) and 21 physically matched healthy controls (control group) were included. Peak power, peak power/kg, average power, and average power were evaluated using the Wingate Anaerobic Test., Results: The peak power (FMF group: 254.8 W [IQR 25/75: 216.4/293.0 W] vs control group: 333.7 W [IQR 25/75: 241.3/570.5 W], p = 0.009), peak power/kg (FMF group: 6.3 W/kg [IQR 25/75: 5.2/7.0 W/kg] vs control group: 7.0 W/kg [IQR 25/75: 6.1/8.6 W/kg], p = 0.046), average power (FMF group: 186.0 W [IQR 25/75: 164.3/211.2 W] vs control group: 231.8 W [IQR 25/75: 181.8/338.1 W], p = 0.006), and average power/kg (FMF group: 4.5 W/kg [IQR 25/75: 3.8/5.0 W/kg] vs control group: 5.1 W/kg [IQR 25/75: 4.2/5.9 W/kg], p = 0.040) were found significantly higher in the control group compared with FMF group., Conclusions: Children with FMF seems to have diminished anaerobic exercise capacity compared with their healthy peers., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

This study compared the hemodynamic changes in the prefrontal cortex during sprint interval training (SIT) and recovery periods in sedentary and athletes. SIT was performed on a cycling ergometer on 12 male athletes and 9 sedentary participants. A functional near-infrared spectroscopy (fNIRS) device was used to record the hemodynamic changes of the prefrontal cortex throughout the protocol. The oxyhemoglobin (Oxy-Hb) levels in the prefrontal cortex were increased significantly, and the power outputs were decreased in repetitive Wingate anaerobic tests (WAnTs) in Sedentary and Athletes group (p < 0.001). In addition, the Sedentary group had higher Oxy-Hb values (p < 0.001). However, the recovery times decreased significantly after all WAnTs (p < 0.05). Despite the increased fatigue, athletes performed better with less Oxy-Hb than the sedentary participants. Also, the recovery of the Oxy-Hb values in the prefrontal region was faster in athletes. These results may highlight a possible brain adaptation in athletes.

Introduction and Objective: Asthma, is the most common chronic inflammatory disease in childhood period. It can affect the daily life to an advanced level and may become vital. The purpose of this study is to compare physical fitness and anaerobic capacity in asthmatic children (AC) and non-asthmatic children (NC)., Materials and Methods: A total of 47 children participated in the study; 25 individuals with mild to moderate asthma and 22 healthy children were assessed. The assessed variables consist pulmonary function and peripheral muscle strength (PMS). Additionally, physical fitness was evaluated by using fitnessgram test battery, which includes body composition, modified shuttle walk test (MSWT), curl-up test, push-up test, and sit and reach test variables. Anaerobic capacity was measured with wingate anaerobic capacity test (WAnT) and counter-movement jump (CMJ) using a tri-axial accelerometer., Results: FEV 1 /FVC ratio (p = 0.01), MSWT (p = 0.001), push-up test (p = 0.01), and WAnT peak power (p = 0.05) were measured significantly to be found reduced in AC compared with that of NC. Between the two groups, PMS, curl-up test, sit and reach test, and CMJ were not significantly different (p = 0.05). High to moderate positive correlation was found among WAnT, CMJ parameters and FEV1, fat-free body mass (FFM), dominant handgrip, and quadriceps strengths (p = 0.05)., Conclusions: Physical fitness level and anaerobic capacity were lower in AC compared with that of NC. Physical fitness parameters and anaerobic exercise capacity should be evaluated on the physiotherapy and rehabilitation program in AC.

A 14-year-old boy presented with sharp left ankle pain for 1.5 years. Evaluation showed an irregular lytic lesion with surrounding sclerosis (diameter, 1.3×1.2×1.1 cm) in the central part of the distal tibial epiphysis, extending from the physis to the plafond with articular penetration. Treatment included intralesional curettage and bone grafting through a mid-anterior epiphyseal bone tunnel with fluoroscopic guidance and use of a gelatin foam sponge to help contain the bone graft within the epiphyseal cavity. Microscopic examination of the excised tissue was consistent with chondroblastoma. Ankle arthroscopy 11 months later showed that the tibial plafond articular surface was fully healed. Two years postoperatively, the patient was asymptomatic and had no recurrence. This case shows that intralesional curettage and bone grafting, with a gelatin foam sponge to prevent graft extrusion, may be used successfully in treating distal tibial epiphyseal chondroblastoma that penetrates the ankle joint. [Orthopedics. 2019; 42(4):e391-e394.]., (Copyright 2019, SLACK Incorporated.)

Exercise capacity has been reported to be lower in children with Juvenile Idiopathic Arthritis (JIA). Therefore, the aim was to investigate the effects of an 8-week water-based exercise program on exercise capacity in children with JIA. Forty-two children with JIA were divided into two groups as: exercise group [n = 21, water-running, moderate-intensity exercise (60-70%), two times/week], and control group (n = 21, no additional treatment other than the prescribed medication). All children were assessed at baseline and post-intervention in terms of physical and disease-related characteristics, pain at rest and in activity (visual analog scale), range of motion (Escola Paulista de Medicina Range of Motion Scale), aerobic exercise capacity (cycle ergometer), and anaerobic exercise capacity (Wingate Test). Anaerobic exercise capacity was found to be improved in the exercise group [baseline: 5.54 W/kg (IQR 25/75: 4.07/6.88 W/kg) vs. post-intervention: 6.0 W/kg (IQR 25/75: 4.8/7.4 W/kg), p = 0.002], while no improvements were observed in the control group [baseline: 5.29 W/kg (IQR 25/75: 4.75/5.85 W/kg) vs. post-intervention: 5.5 watts/kg (IQR 25/75: 5.0/6.1 W/kg), p = 0.076]. The amount of the changes related to anaerobic exercise capacity were higher in the exercise group [exercise group: 0.6 W/kg (IQR 25/75: 0.3/1.3 W/kg) vs. control group: 0.2 W/kg (IQR 25/75: - 0.1/0.5 W/kg), p = 0.024]. No changes were detected related to aerobic exercise capacity in any of the groups (p > 0.05). An 8-week water-running program might be beneficial to improve anaerobic exercise capacity, but it is not enough to improve the aerobic exercise capacity in children with JIA.

Fetal stem cells are a unique type of adult stem cells that have been suggested to be broadly multipotent with some features of pluripotency. Their clinical potential has been documented but their upgrade to full pluripotency could open up a wide range of cell-based therapies particularly suited for pediatric tissue engineering, longitudinal studies or disease modeling. Here we describe episomal reprogramming of mesenchymal stem cells from the human amnion to pluripotency (AM-iPSC) in chemically defined conditions. The AM-iPSC expressed markers of embryonic stem cells, readily formed teratomas with tissues of all three germ layers present and had a normal karyotype after around 40 passages in culture. We employed novel computational methods to determine the degree of pluripotency from microarray and RNA sequencing data in these novel lines alongside an iPSC and ESC control and found that all lines were deemed pluripotent, however, with variable scores. Differential expression analysis then identified several groups of genes that potentially regulate this variability in lines within the boundaries of pluripotency, including metallothionein proteins. By further studying this variability, characteristics relevant to cell-based therapies, like differentiation propensity, could be uncovered and predicted in the pluripotent stage.

Hypoxia causes erythrocyte sickling in vitro; however, its role in the pathophysiology of sickle cell disease is poorly understood. We report that hypoxia rapidly decreased oxygen saturation in transgenic sickle cell disease mice, but this effect was immediately buffered by a robust ventilatory response. The initial hypoxemia improved steadily throughout the duration of hypoxia without any detectable acute pulmonary adverse effect. Furthermore, the mice suffered acute anemia that ironically was associated with lowering of both plasma hemoglobin and heme. These results were corroborated by increased plasma haptoglobin and hemopexin levels. Markers of ischemic tissue injury increased spatiotemporally following repeated hypoxia exposures. This variation was supported by organ-specific induction of hypoxia-responsive genes. Our results show that hypoxia exerts diametric effects on sickle cell disease by promoting ischemic injury while enhancing the expression of hemolysis scavenger molecules. This phenomenon may help to understand the disparate clinical syndromes associated with hemolysis and vaso-occlusion in sickle cell disease., (© 2016 by the Society for Experimental Biology and Medicine.)

Although the endocrine pancreas appears to play an important role in the pathophysiology of sickle cell disease, very little is known about the morphologic changes in this tissue. Our study was initiated to delineate the microscopic features of the endocrine pancreas in a large autopsy series of sickle cell hemoglobinopathies. From more than 650 cases archived at the Centralized Pathology Unit for Sickle Cell Disease (Mobile, AL), 224 autopsy cases were identified for review of clinical and gross autopsy findings and/or for microscopic studies, including histochemical stains (trichrome, reticulin, iron), and immunohistochemical stains (insulin, glucagon, somatostatin, and pancreatic polypeptide). The gross examinations were recorded as unremarkable in 65% of the autopsies. In childhood and adolescence (< or = 18 years), pancreas weights (50.76 +/- 5.16SE gm) were significantly greater (p < 0.0001) than age-matched controls (30.42 +/- 3.59SE gm). In adulthood, pancreas weights (108.34 +/- 5.29SE gm) were not significantly different from controls (110 gm). Microscopic findings included vascular congestion (48%), edema (65%), siderosis (31%), and nesidioblastosis (76%), which included islet cell dispersion (53%), hyperplasia (23%), and hypertrophy (25%). Analysis by age groups suggested that islet cell dispersion/hyperplasia persists unchanged, whereas diameters of compact islets tend to increase with age. These findings may be related to local tissue hypoxia and/or increased metabolic energy needs in sickle cell disease.

Background: The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome., Methods: In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses., Results: Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths., Conclusions: Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.

Most frequently, placental glycogen has been studied as an index of fetal nutrition. There are no published studies of placental glycogen as an index of fetal stress. In this study of 1573 samples from 71 placentae, glycogen levels in the placental disk, fetal membranes and umbilical cord of normal uncomplicated pregnancies were compared with those in complicated pregnancies. The complicated pregnancies included preterm delivery, hypertensive disorders, inadequate prenatal care, substance abuse, maternal fever or infection, obesity, diabetes mellitus, premature rupture of membranes, intrauterine growth retardation, sickle cell trait, and acute meconium staining of amniotic fluid at delivery. The data showed that the only significant differences were in the subgroup complicated by meconium-stained amniotic fluid in which the placental disks and umbilical cords had significantly lower (P=0.0006) glycogen levels. This finding suggests a relatively specific association. It is interesting to speculate that the passage of meconium with its vasoconstrictive effect increases utilization of local glycogen stores, decreases local glycogen reserves needed for the work of further vasoconstriction, and, in the event of subsequent acute stress, impairs vascular perfusion of tissues. In this way, meconium could predispose the infant to asphyxia.

A case is reported of a previously healthy 52-year-old African American male who presented with acute onset of abdominal pain. Progressive increase in his abdominal symptoms led to an exploratory laparotomy; however, no pathology was discovered. Postoperatively, the patient became hypoxemic which progressed to diffuse infiltrates on chest x-ray, suggestive of adult respiratory distress syndrome. He had a rapidly fatal course. Autopsy showed bone marrow infarction, fat embolism, splenomegaly, and widespread congestion with sickle erythrocytes. Hemoglobin electrophoresis done postmortem showed hemoglobin (Hb) SC disease that was undiagnosed antemortem. To the best of our knowledge, it is unusual for Hb SC to be diagnosed postmortem in adults. This case suggests that sickle cell disorders should be ruled out in patients at risk for hemoglobinopathy in the presence of signs and symptoms compatible with the disease, irrespective of age.

Goal: To determine whether children with sickle cell disease (SCD), but without clinical evidence of cerebrovascular disease, have vasculopathy shown by quantitative magnetic resonance angiography (MRA)., Methods: In a retrospective review of MRA films, we compared 47 SCD patients with 49 control patients. Time-of-flight three-dimensional T1-weighted gradient-echo images were reconstructed, by maximum-intensity projection, to show the basilar artery in coronal view, and basilar volume was calculated from measurements made on films. Basilar volume was correlated with hematocrit and with results of cognitive testing., Findings: Mean basilar artery volume was 74% larger in SCD patients than in controls (P<.001). If the upper limit of normal is defined as mean adult volume +2 SD (< or =427 mm(3)), 2% (1 of 43) of controls but 37% (17 of 46) of SCD patients exceed this value (chi(2)=19.0; P<.001). Basilar volume correlated inversely with hematocrit (r=-.60; P<.0001), with full-scale IQ (r=-.62; P<.005), and with freedom from distractability (r=-.61; P<.006) in SCD patients. Analysis of basilar artery tissue from a 5-year-old SCD patient showed that basilar dilatation can be associated with pathological changes typical of hypertension., Conclusions: Approximately 37% of a heterogenous group of pediatric SCD patients had ectasia of the basilar artery. Quantitative MRA is sensitive to subtle vasculopathy that can go undetected in the qualitative analysis more commonly done. Data suggest that there is a substantial elevation of arteriolar blood volume in pediatric SCD patients, and that such patients may share disease features in common with adult hypertension.

To create mice expressing exclusively human sickle hemoglobin (HbS), transgenic mice expressing human alpha-, gamma-, and betaS-globin were generated and bred with knockout mice that had deletions of the murine alpha- and beta-globin genes. These sickle cell mice have the major features (irreversibly sickled red cells, anemia, multiorgan pathology) found in humans with sickle cell disease and, as such, represent a useful in vivo system to accelerate the development of improved therapies for this common genetic disease.

Purpose: The purpose of this report is to describe the clinical and pathologic features of a patient with acute thrombosis of both internal carotid arteries leading to death., Methods: This is a case report of special interest because of extensive brain vessel pathologic examination., Results: The analysis of this case showed that the brain had suffered massive infarction and cerebral edema. The internal carotid arteries (ICAs) were occluded by acute thrombus. The arterial wall of the left ICA, studied at its distal segment, showed a small amount of intimal hyperplasia which did not cause encroachment on the lumen. Immunohistochemical stains indicated that this lesion was formed by proliferative vascular smooth muscle rather than incremental thrombus formation., Conclusion: Acute thrombus formation can occur in the large cerebral arteries of children with sickle cell disease in the presence of only minimal intimal hyperplasia. The intimal hyperplasia which forms the sickle related vasculopathy seen on angiography or detected by Transcranial Doppler may be more related to stimulation of smooth muscle cells than dysregulation of thromboregulation at the endothelial surface. Implications for preventive treatment are discussed.

The Placental Pathology Practice Guideline Development Task Force, a multidisciplinary group, has prepared this guideline to assist those involved with placental examination. It provides recommendations related to indications and methods for placental examination as well as sample worksheets. An algorithm for the handling of placentas summarizes the recommendations of the guideline. A summary of specific findings of placental examination together with their pathogenesis and clinical associations is also provided. Recommendations related to reporting with sample reporting formats are included. The guideline is intended as an educational tool, and its use should be guided by the individual circumstances and care setting of specific cases.

Glycogen in the placenta and its appendages is important for fetal well-being. The precise location of the glycogen stores, however, is unknown. This study was initiated to quantitate glycogen levels at well-defined sampling sites in more than 641 samples from 10 uncomplicated pregnancies and to correlate these glycogen levels with clinical and morphological variables. By biochemical assay, glycogen levels were greatest in the midumbilical cord section (29.08 +/- 1.18 mg/g dry wt) and lowest in the amnionic membrane (2.31 +/- 0.08 mg/g dry wt). Within the placental disk, parenchymal glycogen levels were greatest near the cord insertion (9.31 +/- 2.68 mg/g dry wt) and lowest at the periphery (5.71 +/- 1.14 mg/g dry wt). The midumbilical cord glycogen level showed strong direct correlations (P < .001) with birth weight, umbilical cord weight, and total calculated umbilical cord glycogen and somewhat lower but significant (P < .037) direct correlations with the calculated mean umbilical cord glycogen level, total calculated placental glycogen content, and placental weight. The glycogen level in the middisk parenchymal section from the fetal surface correlated directly with gestational age. Periodic acid-Schiff stains showed that magenta glycogen granules were most abundant in the cytoplasm of the vascular smooth muscle cells. These data show significant variations in glycogen levels among sampling sites. Definition of the precise sampling site is important for clinicopathologic studies of placental glycogen and for interstudy correlations.

Intradural extension of a sacrococcygeal teratoma (SCT) is extremely rare and only well-documented in presacral tumors that have been associated with a familial history, anorectal stenosis, and sacral dysraphism. This case documents the extension of a type I SCT into the dural sac with attachment to the filum terminale. A full-term female was transferred to our tertiary newborn intensive care unit with a sacral mass measuring 12 x 13 cm. It protruded from the buttocks and displaced the anus anteriorly. Rectal examination showed no presacral component. Radiographs demonstrated calcification in the soft tissue mass and a normal-appearing sacrum with the last sacral segment not visualized. At operation during dissection of the cephalad component, the SCT extended into the spinal canal. Neurosurgical consultation resulted in a sacral laminectomy which revealed the tumor to be attached to the tip of the filum terminale. The tumor was removed in toto with all sacral roots preserved. The infant required a second operation to revise a wound dehiscence and suspected cerebrospinal fluid leak. The final pathology report was benign SCT. Follow-up at 2 years showed no recurrence, normal sphincter tone, and a normal computed tomography scan. This represents the first well-documented intradural extension of a Type I SCT with attachment to the spinal cord. This extremely rare occurrence requires awareness with the availability of neurosurgical support to expedite operative management.

Collagenous colitis, a disorder characterized by increased subepithelial collagen deposition associated with an inflammatory infiltrate in the lamina propria, has been reported infrequently in children. An 8-year-old girl with collagenous colitis is described who presented with chronic watery diarrhea and abdominal pain. Biopsy specimens of the colonic mucosa showed the pathological features of collagenous colitis. The patient's symptoms resolved following corticosteroid therapy. Collagenous colitis should be considered in the differential diagnosis of children with chronic diarrhea.

Archival autopsy studies of sickle cell disease have often been hampered by inadequate documentation of the genotype. Although the polymerase chain reaction (PCR) has been applied to the prenatal diagnosis of sickle cell disease, its use has not been reported in archival studies of sickle cell disease. In this study, DNAs from formalin-fixed, paraffin-embedded archival tissues were amplified by PCR and analyzed by dot-blot hybridization using allele-specific oligonucleotides. These S and C genotypes for 9 of 10 archival specimens studied blindly were correctly identified by PCR. The tenth specimen consistently failed to amplify by PCR, yielding no result. These data demonstrate the utility of PCR for retrospective identification of the genotype of sickle cell disease. This application of PCR will significantly expand the number of autopsy cases suitable for retrospective studies of the morbidity and mortality of sickle cell disease.

The hemodynamics of critical aortic stenosis in the fetus make it a ductus-dependent cardiac defect because the ductus arteriosus supplies blood not only to the descending aorta but also to the aortic arch and coronary vessels. In utero closure of the ductus arteriosus has been reported in association with tetralogy of Fallot, truncus arteriosus, maternal use of prostaglandin inhibitors, and as idiopathic events. This is the first report of a ductus-dependent congenital heart defect (critical aortic stenosis) where treatment with indomethacin, a prostaglandin synthetase inhibitor, precipitated premature closure of the ductus and hydrops fetalis. Review of reported cases of premature closure of the ductus show that acute, in utero closure of the ductus in a fetus with limited cardiopulmonary reserves has a worse prognosis than with previously reported cardiac anomalies. This study strongly supports published concerns of increased perinatal morbidity and mortality when fetuses are exposed to prostaglandin inhibitors in utero, and shows that ductus-dependent fetal cardiac defects are contraindications to the maternal use of prostaglandin inhibitors during pregnancy.

A 21-day-old infant presented with anemia, conjugated hyperbilirubinemia, hypoproteinemia, and a severe coagulopathy. The hospital course was marked by progressive hepatic failure, encephalopathy, and renal insufficiency. The infant died on day 15 of hospitalization. Postmortem examination showed diffuse hepatic fibrosis and marked siderosis of the liver, pancreas, kidney, adrenal glands, and the duodenal epithelium, with sparing of the reticuloendothelial system. These findings were characteristic of idiopathic neonatal iron-storage disease. Previously reported cases are summarized and discussed. An increased awareness and understanding of this rapidly fatal disorder will be important for genetic counseling and possibly in defining an aberrant mechanism in the handling of iron.

Ischemia-reperfusion injury has been implicated as playing a major role in the development of necrotizing enterocolitis, a major cause of morbidity and mortality in the newborn. A tungsten-supplemented molybdenum-free diet can reduce xanthine oxidase (XO) enzyme activity in the intestine, which in turn reduces the generation of oxygen radicals after an ischemia-reperfusion insult. This study evaluated the ability of this diet to be effective by indirect means, ie, transplacental and breast-feeding routes. XO activity of the intestine was measured in three groups of CD-1 white rats: I, weanlings fed the tungsten diet or standard chow for 1 week; II, 1-day-old rat pups whose mothers were maintained on the tungsten or standard chow for 7 to 10 days prior to term; and III, rat pups at 1 and 3 weeks after birth whose lactating mothers were maintained on the tungsten or standard chow. Some animals from group III also underwent either a 30- or 60-minute episode of occlusion of the superior mesenteric artery (SMA) to evaluate the protective effects of the diet. XO activity was significantly reduced in all groups receiving the tungsten diet (P less than .0001). Blinded histopathologic studies of the entire small bowel showed significantly less villar necrosis (P less than .05) and fibrosis (P less than .0001) in the tungsten-treated group than in the controls. In the 60-minute occlusion study all tungsten-group animals survived, whereas 7 of 12 in the control group died of intestinal infarction within 24 hours (P less than .001).(ABSTRACT TRUNCATED AT 250 WORDS)

In a hamster model of non-Hodgkin's lymphoma which closely parallels the disease in man, and which is induced by an unusual agent(s), a diarrheal bowel disease was a major cause of mortality. This study was initiated to characterize this bowel disease and its relation to lymphoma induction and to natural diseases seen in the hamster. The studies showed that the bowel disease was an ulcerative process and was distinct from natural diseases. The incidence of the bowel disease correlated directly with that of the lymphoma in repeated epizootics, in titration studies, and in agent inactivation tests. The ulcerative bowel lesions were seen at the same stage of the disease as acute and chronic inflammatory infiltrates with necrosis in the thymus and mesenteric lymph nodes. Since necrosis in the gut-associated lymphoid tissue can lead to perforation and sepsis, these bowel lesions were lethal, whereas similar necrosis in other lymphoid tissues (thymus and lymph nodes) could be clinically undetectable. Similar lesions have been reported in man. The ulcerative bowel disease was a reliable early clinical marker for exposure of hamsters to this lymphomagenic agent(s).

A patient with sickle cell disease and systemic necrotizing vasculitis is reported. Although there are a number of immune defects in patients with sickle cell disorders, this association has not been previously reported. We suspect that immunologic disorders frequently are unrecognized in these patients because the symptoms are attributed to complications of sickle cell disease.

Thirty-two patients who had polypoid melanoma were identified in a registry of 552 melanoma patients. The tumor is regarded as a variant of nodular melanoma and is associated with an increased thickness, more frequent ulceration than the nodular variant of melanoma, younger patient age, and higher probability of occult metastasis. Polypoid melanomas were most frequently present on the trunk, and were also encountered in unusual sites, such as the mucosa of the nose, hard palate, and anorectal junction. In terms of survival, the patients with the polypoid nodular variant fared significantly worse than those with nonpolypoid nodular (P = 0.05) and those with superficial spreading (P = 0.003) melanomas. The five-year survival rate for polypoid variant was 42%, in contrast to 57% for the nonpolypoid nodular and 77% for the superficial spreading melanomas. The poor prognosis of patients who have polypoid melanoma is most likely due to its being the type of melanoma with the deepest penetration at the time of surgical excision.

This Latin dissertation on diseases of the prostate gland was written by the German physician Maximillian J.F. Bruch at the Freiderick Wilhelm University. This English translation permits a rare glimpse into the early medical thinking on prostatic diseases.

This study investigated the influence of the location of the sampling site during elemental analyses of 21 human term placentae. The levels of iron, zinc, copper and calcium in fetal membranes, umbilical cords and placental discs were measured by atomic absorption spectrophotometry and compared. The disc samples were obtained from central (peri-insertion and mid-disc fetal and maternal halves), and peripheral regions. Significant variations were found. Copper was present in highest levels (17.2 +/- 2.0 micrograms/g dry weight) in the fetal membranes. Calcium levels were highest (712 +/- 47 micrograms/g dry weight) in the periphery of the placental disc. Iron levels were highest (558 +/- 14 micrograms/g dry weight) in the central regions of the disc. Zinc levels were lower (50.3 +/- 1.4 micrograms/g dry weight) in the fetal half of the mid-disc regions than in the maternal half (56.0 +/- 1.2 micrograms/g dry weight). This study demonstrates the importance of defining the location of the sampling site in studies involving elemental analysis of the placenta.

Although many studies in animal models and in cell cultures have shown that vanadate has insulin-like effects, it has not been studied in human diabetes mellitus. In this study the levels of vanadium in human placentae from 23 pregnancies complicated by gestational diabetes mellitus were compared with 18 uncomplicated non-diabetic pregnancies closely matched for maternal age, gravidity, and gestational age. Using the unpaired Student's t-test, the mid-disc placental levels in gestational diabetes (7.62 +/- 1.29 micrograms/g dry weight) were significantly lower (p less than 0.05) than controls (8.73 +/- 1.85 micrograms/g dry weight). These findings appear to be independent of placental size and birthweight. When these data were analyzed according to treatment, the vanadium levels in insulin-treated cases (8.07 +/- 1.32 micrograms/g dry weight) were not significantly different from the matched controls (8.84 +/- 1.69 micrograms/dry weight); the levels in noninsulin treated cases (7.08 +/- 1.25 micrograms/g dry weight), however, were significantly (p less than 0.005) lower than controls (8.99 +/- 1.96 micrograms/g dry weight). It is interesting to speculate that there may be increased binding of vanadium to maternal tissues in human diabetes mellitus when insulin is deficient.

The osteoclastic giant cell tumor of the pancreas is a rare primary neoplasm that by light and electron microscopy mimics giant cell tumor of bone. In the proper clinical setting, this lesion can be diagnosed by fine needle aspiration. Review of 10 cases reveals a female predominance, a median survival of 12 months, and a tendency for local invasion, rather than lymphatic or distant metastasis, and for invasion of large veins. An epithelial origin is favored for this malignant neoplasm, which expresses varying degrees of mesenchymal differentiation.

Recent studies have implicated oxygen free radicals in ischemia-reperfusion injury to the gastric mucosa. The aims of the present study were to test the hypothesis that the enzyme xanthine oxidase is the source of the oxygen radicals in the ischemic stomach and determine the importance of the iron-catalyzed Haber-Weiss reaction in generating the cytotoxic oxygen radicals. Gastric mucosal clearance of 51Cr-labeled red blood cells was measured during a 30-min control period, a 30-min ischemic period (hemorrhage to 25 mmHg arterial pressure), and a 60-80-min reperfusion period (reinfusion of shed blood). In untreated (control) rats, a dramatic rise (100-fold) in the leakage of 51Cr-labeled red blood cells into the gastric lumen was observed only during the reperfusion period. After the reperfusion period, gastric mucosal damage was further assessed using gross lesion area and histology. Rats were placed on a sodium tungstate diet (to inactivate xanthine oxidase), or treated with either deferoxamine (an iron chelating agent) or superoxide dismutase (a superoxide scavenger). All three interventions substantially reduced 51Cr-labeled red blood cell clearance and gross lesion area relative to untreated rats. However, tissue injury assessed histologically was similar in both treated and untreated animals. The results of this study support the hypothesis that oxygen free radicals mediate the hemorrhagic shock-induced extravasation of red blood cells. The data also indicate that xanthine oxidase is the source of the oxy-radicals and that the iron-catalyzed Haber-Weiss reaction is largely responsible for hydroxyl radical generation in this model.

The aim of the present study was to determine whether the split products of sulfasalazine, sulfapyridine, and 5-aminosalicylic acid can ameliorate ischemia/reperfusion-induced injury to the gastric mucosa. Gastric mucosal damage was assessed by measuring (a) 51Cr-labeled red blood cell leakage into the gastric lumen, (b) the area of gross mucosal lesions, and (c) the extent of histologically demonstrable mucosal damage. In rats treated with 5-aminosalicylic acid, but not in those treated with sulfapyridine, the leakage of 51Cr-labeled red blood cells and the area of gross mucosal lesions after ischemia/reperfusion were significantly reduced as compared with untreated (control) rats. Inasmuch as 5-aminosalicylic acid (the therapeutic moiety of sulfasalazine) has been reported to be a hydroxyl radical scavenger, we also assessed the effects of dimethylsulfoxide (another hydroxyl radical scavenger) on ischemia/reperfusion-induced gastric mucosal injury. In rats treated with dimethylsulfoxide, leakage of 51Cr-labeled red blood cells and the area of gross mucosal lesions after ischemia/reperfusion were significantly reduced as compared with control rats. The results of this study support the contention that ischemia/reperfusion-induced gastric bleeding involves the hydroxyl radical and indicate that 5-aminosalicylic acid significantly attenuates this vascular injury.