Three cases of alloimmune mediated pancytopenia in calves resembling bovine neonatal pancytopenia.

BACKGROUND: Between 2007 and 2011 several thousands of calves died from bovine neonatal pancytopenia (BNP), a bleeding syndrome triggered by vaccine induced alloantibodies from the dams. Following withdrawal of the involved bovine viral diarrhoea virus (BVDv) vaccine, the incidence of this condition rapidly decreased, with no reported cases in the last 5 years. Here, we report a recent immune-mediated pancytopenia in three calves from two different suckler herds, clinically indistinguishable from BNP. CASE PRESENTATION: Three Belgian Blue suckler calves from two different farms, aged around two weeks, showed multiple bleedings disseminated on the skin and petechiae and ecchymoses on the mucosae. Blood examination confirmed anaemia, leukopenia and thrombocytopenia. BVDv infection was excluded. Despite blood transfusion and cortisone therapy, all three animals died. Necropsy and histology confirmed bone marrow depletion. Binding of IgG from the dams on leukocytes of the calves was demonstrated by flow cytometry. Two calves, originating from the same farm, received colostrum from the same dam. None of the calves were given colostrum replacers or colostrum supplements. No link with the BNP causing BVDv vaccine could be evidenced. However, dams had been vaccinated against bovine herpesvirus 1, parainfluenza-3 virus, bovine respiratory syncytial virus and bluetongue virus serotype 8. CONCLUSIONS: Alloimmune mediated pancytopenia was evidenced in three animals, clinically and pathologically indistinguishable from BNP. Whether this disease is again vaccine mediated remains to be determined.

Remarkable geographical variations between India and Europe in carriage of the staphylococcal surface protein-encoding sasX/sesI and in the population structure of methicillin-resistant Staphylococcus aureus belonging to clonal complex 8.

OBJECTIVES: sasX is a colonization-virulence factor that potentially underlies the success of methicillin-resistant Staphylococcus aureus (MRSA) sequence type (ST) 239 in Asia. We aimed to study the spread of sasX and the population structure of MRSA in two geographically distinct regions, Europe and India. METHODS: MRSA (n = 128) from screening and clinical samples from tertiary care patients in 12 European countries (n = 119), and from India (n = 9) were multilocus-sequence-typed and screened for sasX and its carrier φSPß-like prophage by PCR. Whole genome sequencing was performed on sasX-harbouring strains from India (n = 5) and Europe (n = 2) and on a selection non-harbouring sasX (n = 36) (2 × 150 bp, Miseq, Illumina). Reads were mapped to the ST239 reference strain, TW20. RESULTS: sasX and sesI, a sasX homologue native to Staphylococcus epidermidis, were detected in five of the nine Indian MRSA belonging to ST239 and to other sequence types of CC8. In contrast, sasX was restricted to two ST239 strains in Europe. The intact sasX and sesI carrier φSPß-like prophages were ∼80 kb and ∼118 kb, and integrated in the yeeE gene. We identified 'novel' ST239 clades in India and Serbia that showed significant differences in base substitution frequencies (0.130 and 0.007, respectively, Tamura-Nei model) (p <0.05). CONCLUSIONS: Our data highlight dissemination of sasX to non-ST239 sequence types of CC8. Detection of the S. epidermidis-associated sesI in MRSA provided unquestionable evidence of transfer between the two species. Stark differences in evolutionary rates between the novel Indian and Serbian ST239 clades identified here might be due to inherent clade characteristics or influenced by other environmental differences such as antibiotic use.

Multidetector computed tomography diagnosis of gastric volvulus through the foramen of Morgagni.

Morgagni hernia is considered to be the rarest form of all diaphragmatic hernias. It develops through a congenital defect in the retrosternal area. Usually asymptomatic, this entity can lead to life-threatening complications such as incarceration, strangulation or volvulus of the herniated viscus. We hereby report a rare case of organoaxial gastric volvulus producing through the foramen of Morgagni in a 78-year-old woman. The full diagnosis was made by upper gastro-intestinal series and multidetector computed tomography (MDCT). The basic anatomy, physiopathology, diagnostic methods, complications and surgical treatment of Morgagni hernia are briefly reviewed.

Familial adenomatous polyposis: clinical presentation, detection and surveillance.

Colorectal cancer (CRC) is a leading cause of cancer related death in the western countries. It remains an important health problem, often under-diagnosed. The symptoms can appear very late and about 25% of the patients are diagnosed at metastatic stage. Familial adenomatous polyposis (FAP) is an inherited colorectal cancer syndrome, characterized by the early onset of hundred to thousands of adenomatous polyps in the colon and rectum. Left untreated, there is a nearly 100% cumulative risk of progression to CRC by the age of 35-40 years, as well as an increased risk of various other malignancies. CRC can be prevented by the identification of the high risk population and by the timely implementation of rigid screening programs which will lead to special medico-surgical interventions.

Staged use of the transsphenoidal approach to resect superior third ventricular craniopharyngiomas.

INTRODUCTION: Craniopharyngiomas are benign tumors, usually originating from the infundibulum or tuber cinereum. Their surgical treatment is challenging because of their relationship to neural and vascular structures. Large craniopharyngiomas that invade the upper third of the third ventricle are a common reason for patients to need a second operation to accomplish a gross total resection. Transsphenoidal approaches are being increasingly used in the treatment of craniopharyngiomas. Large craniopharyngiomas involving the superior third ventricle are most commonly resected through a staged approach, often involving a transcortical or interhemispheric route. CASE REPORT: The authors describe the use of an extended transsphenoidal approach as a second-stage operation to resect the intraventricular component of a large craniopharyngioma in an illustrative case. CONCLUSION: The authors find this to be an excellent indication for an endoscopic extended transsphenoidal approach in selected cases.

Increased frequency of CD16+ monocytes and the presence of activated dendritic cells in salivary glands in primary Sjögren syndrome.

OBJECTIVES: In the salivary glands of patients with primary Sjögren Syndrome (pSjS) an accumulation of dendritic cells (DCs) is seen, which is thought to play a role in stimulating local inflammation. Aberrancies in subsets of monocytes, generally considered the blood precursors for DCs, may play a role in this accumulation of DCs. This study is aimed at determining the level of mature CD14lowCD16+ monocytes in pSjS and their contribution to the accumulation of DCs in pSjS. METHODS: Levels of mature and immature monocytes in patients with pSjS (n = 19) and controls (n = 15) were analysed by flow cytometry. The reverse transmigration system was used for generation of DCs generated from monocyte subsets. The phenotype of DCs in pSjS salivary glands was analysed using immunohistochemistry. In vivo tracking of monocyte subsets was performed in a mouse model. RESULTS: Increased levels of mature CD14lowCD16+ monocytes were found in patients with pSjS (mean (SD) 14.5 (5.5)% vs 11.4 (3.4)%). These cells showed normal expression of chemokine receptor and adhesion molecules. Mature monocytes partly developed into DC-lysosome-associated membrane glycoprotein (LAMP)+ (19.6 (7.5)%) and CD83+ (16 (9)%) DCs, markers also expressed by DCs in pSjS salivary glands. Monocyte tracking in the non-obese diabetic (NOD) mouse showed that the homologue population of mature mouse monocytes migrated to the salivary glands, and preferentially developed into CD11c+ DCs in vivo. CONCLUSIONS: Mature monocytes are increased in pSjS and patient and mouse data support a model where this mature monocyte subset migrates to the salivary glands and develops into DCs.

Transient and rapidly resolving intrahepatic portal gas: CT findings.

Hepatic portal gas (HPG) has historically been associated with high (75% or over) mortality rates related to abdominal dramatic conditions and has served as an indicator for urgent exploratory laparotomy. Over the last two decades, the greater availability and higher speed of use of CT as well as improvement in the management of critically ill patients have increased the sensitivity of imaging HPG. HPG has been found associated with a broad range of diseases, some of which are benign and do not necessarily require urgent exploratory laparotomy in the absence of signs of intra-abdominal acute condition or systemic toxicity. We present a case of transient and rapidly resolving HPG found in a 61-year-old male admitted with hypovolemic shock due to upper gastrointestinal occlusion. HPG rapidly resolved after resuscitation of the patient with intensive re-hydration and drastic decompression of the fluid-full stomach through a nasogastric tube. The physiopathology and causes of HPG are briefly reviewed and its potential clinical significance is resituated.

Visual information processing in high-functioning individuals with autism spectrum disorders and their parents.

The authors assessed visual information processing in high-functioning individuals with pervasive developmental disorders (PDD) and their parents. The authors used tasks for contrast sensitivity, motion, and form perception to test visual processing occurring relatively early and late in the magnocellular-dorsal and parvocellular-ventral pathways. No deficits were found in contrast sensitivity for low or high spatial frequencies or for motion or form perception between individuals with PDD in comparison with a matched control group. Individuals with PDD performed equally with or better than controls on motion detection tasks. In addition, the authors did not find differences on any of the tasks between parents of the PDD group and matched control parents. These results indicate that high-functioning individuals with PDD and their parents are able to process visual stimuli that rely on early or late processing in the magnocellular-dorsal and parvocellular-ventral pathways as well as controls.

An increased MRP8/14 expression and adhesion, but a decreased migration towards proinflammatory chemokines of type 1 diabetes monocytes.

In the early development of type 1 diabetes macrophages and dendritic cells accumulate around the islets of Langerhans at sites of fibronectin expression. It is thought that these macrophages and dendritic cells are derived from blood monocytes. Previously, we showed an increased serum level of MRP8/14 in type 1 diabetes patients that induced healthy monocytes to adhere more strongly to fibronectin (FN). Here we show that MRP8/14 is expressed and produced at a higher level by type 1 diabetes monocytes, particularly after adhesion to FN, creating a positive feedback mechanism for a high fibronectin-adhesive capacity. Also adhesion to endothelial cells was increased in type 1 diabetes monocytes. Despite this increased adhesion the transendothelial migration of monocytes of type 1 diabetes patients was decreased towards the proinflammatory chemokines CCL2 and CCL3. Because non-obese diabetic (NOD) mouse monocytes show a similar defective proinflammatory migration, we argue that an impaired monocyte migration towards proinflammatory chemokines might be a hallmark of autoimmune diabetes. This hampered monocyte response to proinflammatory chemokines questions whether the early macrophage and dendritic cell accumulation in the diabetic pancreas originates from an inflammatory-driven influx of monocytes. We also show that the migration of type 1 diabetes monocytes towards the lymphoid tissue-related CCL19 was increased and correlated with an increased CCR7 surface expression on the monocytes. Because NOD mice show a high expression of these lymphoid tissue-related chemokines in the early pancreas it is more likely that the early macrophage and dendritic cell accumulation in the diabetic pancreas is related to an aberrant high expression of lymphoid tissue-related chemokines in the pancreas.

Autofluorescence distribution along the corneal axis in diabetic and healthy humans.

Corneal autofluorescence, as measured with a commercial scanning fluorophotometer (lambda(exc): 415-491 nm; lambda(em): 515-630 nm), is increased in patients with diabetes mellitus. However, such fluorophotometers register an average fluorescence signal over all corneal layers as a consequence of their limited axial resolution of 0.5 mm. In order to determine the location of the fluorophores responsible for the increased corneal autofluorescence measured in diabetics, an attempt was made to measure in vivo the distribution of autofluorescence along the optical axis of the cornea with a modified slitlamp. Fluorescence excitation and emission filters identical to those of the scanning fluorophotometer were fitted to a slitlamp equipped with a slow scan CCD camera. Corneal autofluorescence intensity profiles were obtained with the slitlamp in five patients with severe diabetic retinopathy and compared to those of age-matched healthy controls. Corneal autofluorescence was also measured with the scanning fluorophotometer for comparison. The resolution of the CCD camera for measurement of fluorescence along the corneal axis was 0.1 mm. The corneal autofluorescence intensity of the patients and the healthy controls gradually decreased by about the same amount from the endothelium to the epithelium (57% mm(-1)+/-6 s.d. and 52% mm(-1)+/-5 s.d., respectively). The area under the fluorescence intensity curve was significantly greater for the patients than for the healthy controls (factor 2.4+/-1.0 s.d., P<0.001) and was proportional to the corneal fluorescence measured with the scanning fluorophotometer (r=0.92, P<0.001). The results show that (1) the distribution of autofluorescence along the corneal axis can be measured in vivo in humans, (2) the fluorophores involved are distributed throughout the cornea, and (3) the relative distribution of fluorescence is similar in diabetic patients and healthy controls.

Conversion of lens slit lamp photographs into physical light-scattering units.

PURPOSE: To derive from lens slit lamp photographs by means of densitometry the physically defined quantity for light scattering (the Rayleigh ratio) and to expand the use of the Lens Opacity Classification System (LOCS III) to include clear lenses and also to calibrate the LOCS III Nuclear Opacity (NO) score in physical terms. METHODS: Series of slit lamp photographs were taken from 38 eyes from 29 subjects (age range 18 to 84 years old) including cataracts, for 0.1- and 0.2-mm slit width, using 200 ASA and 1600 ASA film speed (Kodak professional; Eastman Kodak, Rochester, NY) and different flash settings with a Topcon SL-6E (12 slit/speed/flash combinations; Paramus, NJ). Additionally 19 eyes were photographed with a Zeiss 40 SL/P (8 slit/speed/flash combinations; Carl Zeiss, Thornwood, NY). A calibrated suspension of latex spheres also was photographed at the same 20 conditions. Densitometry was performed on the nuclear area of all photographs including the LOCS III standards, using a photometrically corrected photocell. Slit width and flash intensity settings were photometrically calibrated. All eyes and the suspension were digitally "photographed" with the EAS-1000 (Nidek, Gamagori, Japan) Scheimpflug system. RESULTS: For each eye and the suspension, the series of 20 or 12 densities, corresponding to a range of about 1 log unit in the amount of light used, proved to follow closely a course common to all eyes (the two film characteristics), apart from a shift in the amount of light (because of the differences in light back scattering). CONCLUSIONS: From normal slit lamp photographs, the physical quantity for light (back) scattering can be derived using transformation graphs derived in this study. The LOCS III NO score also can be used for clear lenses and translated into physical units. In this way, slit lamp photography can be used better for more precise studies, provided some minimal calibration of the photograph slit lamp.

[Computed tomographic diagnosis of biliary ileus]. / Diagnostic tomodensitometrique de l'ileus biliaire.

Rare in the general population, gallstone ileus accounts for 25 per cent of nonstrangulated small bowel obstructions in patients over the age of 65. While mortality has declined over the years, it remains high at 12-17 per cent. This is largely due to the insidious symptoms making the diagnosis difficult and to the aged patient population, with frequent comorbid medical conditions contributing to mortality. Much better than plain radiograph and probably easier than ultrasound, CT makes the correct diagnosis of the classical triad of Rigler: distended small bowel loops, pneumobilia and an ectopic calcified gallstone. We report two cases promptly and specifically diagnosed with CT. The second case was a very rare Bouveret's Syndrome, a gastric outlet obstruction caused by a gallstone. Patients were treated by a one time associated enterolithotomy and cholecystectomy. We recommend the early use of abdominal CT scanning for the investigation of clinical bowel obstruction in the elderly, where gallstone ileus is a more common condition.

Postmenopausal osteoporosis: microradiographic aspects.

A comparative microradiographic and histologic analysis of undecalcified bone samples was performed in men and women aged 18-98 years. These morphological methods showed that besides usual lamellar bone remodelling, all the so-called inert surfaces, namely both haversian and vascular canals as well as trabecular surfaces, were involved in weathering alterations of the superficial lamellae, resulting in eroded outlines devoid of osteoclast. These aspects, recorded in all pieces of our material, were visible from the earliest adult age and were randomly distributed. Except the grade of osteoporosis at a given age, the microradiographic and histologic aspects were similar in both aged men and women and did not allow sex distinction. These observations were consistent with the hypothesis of a particular destructive process affecting all the quiescent lamellar bone surfaces without osteoclast or cell participation. This kind of erosion, termed delitescence, could be at least partially responsible for the age-related and postmenopausal bone loss. In order to explain the increasing osteoporosis after menopause, it has been suggested that the estrogen deficiency could increase the percentage of dead osteocytes. Thereby the reduced cellular control on the bone surface could impair the remodeling process and fail to adapt the bone structure by repairing the microscopic lesions.

[Presentation of an acute and painful giant, necrotic ileal leiomyoma]. / Présentation aiguë et douloureuse d'un leiomyome iléal géant et nécrotique.

We report on a case of giant necrotic ileal leiomyoma presenting uncommonly as a painful pelvic mass mimicking a sigmoid inflammatory process. The classical symptoms and clinical aspects of intestinal leiomyoma are briefly summarized. The CT and US aspects are described with peculiar attention to color Doppler which played an important role in the diagnosis of our patient.

The clinical [corrected] background of familial adenomatous polyposis. History, epidemiology, diagnosis and treatment.

Familial adenomatous polyposis (FAP) is a dominantly inherited genetic disorder predisposing to colon cancer through the early development of multiple adenomatous polyps in the large bowel. FAP is not restricted to the colon and rectum, but is a more complex disease which can potentially affect almost any organ not only with benign tumours but also with life threatening carcinomas. Desmoid tumours and gastroduodenal polyps and cancer are the two more worrying extracolonic manifestations of FAP. Recent advances in FAP knowledge, such as the report of congenital hypertrophy of the retinal pigment epithelium (CHRPE) or the APC gene identification, are very useful for screening and long-term follow-up of the patients through regional or national registries. Nutritional and pharmacological intervention trials are under way to assess potential new medical treatments of FAP. Surgery is still the only effective treatment for colorectal cancer prevention in FAP. The choice of a surgical procedure is controversial, but the introduction of total proctocolectomy with ileal pouch-anal anastomosis can be considered as a major advance in surgical treatment of FAP during the last decade.

[Pulmonary edema secondary to the intravenous administration of iodized contrast. A clinical case with high-resolution tomodensitometry studies]. / Oedème pulmonaire secondaire à l'administration de contraste iodé. Un cas clinique avec analyse tomodensitométrique en haute résolution.

Contrast media related reactions are numerous and of variable gravity. We report a case of pulmonary edema related to the intravenous administration of contrast media. Cardiogenic and non cardiogenic pulmonary edema are pathophysiologic possibilities. High resolution CT findings associate patchy ground glass areas and smooth thickening of subpleural septa, both rapidly disappearing.

[A cause of intestinal intussusception in an adult]. / Une cause d'invagination intestinale chez l'adulte.

Inflammatory fibroid polyp is a very rare tumor involving the gastrointestinal tract and especially the stomach and small bowel. It presents either as a solitary pedunculated or sessile lesion arising from the submucosa. Despite its large size and sometimes very infiltrating growth, the polyp is benign and has a good prognosis. The aetiology remains unknown but it probably represents a reactive proliferation similar in many respects to the granuloma pyogenicum. We report an ileal case which presented itself as an acute intussusception. The very typical and complete sonographic and tomodensitometric findings of this intussusception are detailed. The polyp alone does not have noteworthy radiological characteristic signs and must be included in the large and essentially histological differential diagnostic of gastrointestinal tumors.

[Infected urachal cyst]. / Kyste de l'ouraque surinfecté.

There is a wide variety of pathologies associated with the presence of urachal remnants. We describe a case of infected urachal cyst in a young adult male, with classical symptoms of dysuria, lower abdominal pain, and fever. Ultrasound, cystography and CT are described, ultrasound being often the modality of choice. Differential diagnosis of acute abdominal and pelvic pain or a midline lower abdominal mass at this age should include infection of an urachal remnant.

[Familial adenomatous polyposis: what is new for the clinician?]. / La polypose adénomateuse familiale: quoi de neuf pour le clinicien?

Familial adenomatous polyposis (FAP) is a rare autosomal-dominant disease characterized by the development of more than 100 colorectal adenomatous polyps in young adults. In the absence of surgical intervention, colorectal cancer ineluctably develops in all affected patients. Recent progress in the isolation of the gene responsible for the disease allows to detect gene carriers before they present with symptoms attributable to polyps. Moreover, the presence of four or more lesions of congenital hypertrophy of the retinal pigment epithelium is an extracolonic manifestation of FAP allowing presymptomatic screening of this disease. An effective screening programme combined with the elaboration of a registry for FAP and prophylactic colectomy should reduce mortality related to colorectal cancer. Two other extracolonic manifestations of FAP remain major causes of death: abdominal desmoid tumors and duodenal adenocarcinoma. At this time, no effective medical or surgical therapy has been found to cure these lesions. Restorative proctocolectomy with ileal reservoir is another major advance. This procedure is now regarded as the treatment of choice for patients with FAP because radical removal of all premalignant colorectal mucosa eliminates the risk of subsequent development of a colorectal adenocarcinoma.