Relation of granulomas to lymphatic vessels in Crohn's disease

AIMS: To examine the relation between granulomas and lymphatic vessels in Crohn's disease. METHODS: Formalin fixed, paraffin wax embedded sections were selected from surgical resection specimens from 10 cases diagnosed as Crohn's disease. The block that showed the most granulomas was selected from each case. Sections 5 microns thick were immunostained with antibodies directed against the endothelial markers factor VIII related antigen and Ulex europaeus lectin, and against the vascular wall components collagen IV and laminin. Granulomas were counted on each slide in the serosa, muscularis propria, submucosa, and mucosa. In each area granulomas were classified according to their relation to lymphatic or blood vessels. RESULTS: Overall, an average of 46.1% of granulomas (range 15.3-90.4%) was related to lymphatic vessels, with the majority of these being adjacent to the vessel, rather than in the lumen or distorting the wall. A smaller percentage (10.1%, range 2.4-25.8%) was related to blood vessels. CONCLUSIONS: A significant proportion of granulomas in Crohn's disease is associated with lymphatic vessels. Blood vessel involvement may be a secondary phenomenon, rather than the primary event.     

Liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement

BACKGROUND & AIMS: Hepatic involvement in hereditary hemorrhagic telangiectasia is common but often asymptomatic. However, in some cases, the vascular lesions that involve the liver may lead to high-output cardiac failure and pulmonary hypertension that is predominant over hepatobiliary manifestations. Liver transplantation and treatment of these complications are described and discussed in this article. METHODS: Three patients with hereditary hemorrhagic telangiectasia and hepatic involvement received transplants. They had pulmonary hypertension and chronic right-sided heart failure caused by disseminated intrahepatic telangiectasias with shunts between the hepatic artery and hepatic veins or portal vein. Left-to-right intrahepatic shunt output was estimated to range between 51% and 57.5% of cardiac output. RESULTS: Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheterization performed 6 months later were normal. Follow-up periods currently are 65, 53, and 29 months, and each patient continues to be asymptomatic. CONCLUSIONS: This report suggests that liver transplantation can be considered as an alternative and successful curative treatment that may prevent the irreversible evolution of cardiopulmonary disease.     

Angiographic image of intestinal angiodysplasia of the duodenum in comparison with endoscopy--case report

Angiodysplasia of the bowel wall is a malformation of intestinal giodysplasia of the bowel wall is a malformation of intestinal blood vessels. There seem to be different aetiologies of this disease entity. It is reported to be a common cause of occult intestinal bleeding in patients with negative findings on primary endoscopy of the upper and lower gastrointestinal tract. They occur most frequently in the right colon. The case reported demonstrates endoscopy and arteriography of extensive angiodysplasia in duodenum and proximal jejunum causing severe bleeding.     

Measuring food intake in wild animals: primates

Rabbit hemorrhagic disease is a rapidly lethal infection caused by a calicivirus, characterized by acute liver damage and disseminated intravascular coagulation (DIC). Following morphological criteria and using a specific in situ labeling technique, we have found that liver cell death induced upon infection is due to apoptosis, and that programmed cell death is a constant feature in rabbits experimentally infected with RHDV. The process affected mainly hepatocytes, but also macrophages and endothelial cells presented morphologic hallmarks of apoptosis, expressing all these cell types viral antigens as determined by immunohistochemistry. The occurrence of programmed cell death was correlated with the appearance of the RHDV induced pathology in tissues by DNA fragmentation detection in situ. Hepatocyte apoptosis produced extensive parenchymal destruction causing a lethal, acute fulminant hepatitis that is characteristic of RHD. Apoptosis of intravascular monocytes and endothelial cell was observed together with fibrin thrombi in blood vessels. Since apoptotic cells are known sites of enhanced procoagulant activity, apoptosis of these cell populations might constitute a first step in the pathogenesis of DIC and a common pathway to other viral hemorrhagic fevers. In conclusion, apoptosis in RHD may be determinant in the development of the pathogenesis of this disease.     

Current concepts of tumor-induced angiogenesis

Tumor induced angiogenesis is responsible for the nutrition of the growing tumor and can also increase the probability of hematogenous tumor dissemination. Data obtained from morphological analysis of tumor angiogenesis can contribute to the development of new anti-angiogenic therapies. Based on in vitro and in vivo observations several models of angiogenesis were introduced, explaining the mechanism of lumen formation and the timing of basement membrane depositon. (1) Lumen is formed either by cell body curving or by fusion of intracellular vacuoles of nonpolarized endothelial cells. New basement membrane is deposited after lumen formation. (2) Slit-like lumen is immediately formed by migrating polarized endothelial cells. Basement membrane is continuously deposited during endothelial cell migration, only cellular processes of the endothelial cell migrating on the tip of the growing capillary are free of deposited basement membrane material. (3) Development of transluminal bridges in larger vessels a process called intussusceptive growth leads to the division of the vessels. These models, however, describe angiogenesis in tissues rich in connective tissue. Different processes of angiogenesis take place in organs such as liver, lungs, adrenals, which are the most frequent sites of metastasis having high vessel density without sufficient space for capillary sprouting. In the case of liver metastases of Lewis lung carcinoma the proliferation of endothelial cells was elicited only by direct contact between tumor and endothelial cells, leading to the development of large convoluted vessels inside the metastases. These vessels were continuous with the sinusoidal system, suggesting that these metastases have dual blood supply. This observation, among others, is in contrast to the generally accepted view that liver tumors have arterial blood supply. The increasing number of data demonstrating the dual or venous blood supply of liver metastases should be taken into consideration in the therapy of liver metastasis.     

Paratesticular tumor. Liposarcoma of the spermatic cord

Liposarcoma of the spermatic cord is a rare tumour typically presenting in elderly people. It tends to grow around blood vessels. Most of them have a low level of malignancy and relapses tend to be localized. Metastasis, when present, occur through the haematological route, lymph spread being very unusual. Pre-operative diagnosis is infrequent and overall prognosis is good. The choice treatment is radical orchidectomy, while long-term follow-up of these patients is mandatory. When local relapses become evident, the choice treatment is extensive local excision, followed by optional adjuvant radiotherapy. Chemotherapy has not proven to be effective. This paper reviews the current literature and contributes one case of mixed liposarcoma of the spermatic cord.     

Hemobilia caused by a giant benign hemangioma of the liver: report of a case

We report herein the extremely unusual case of a 39-year-old woman in whom a giant cavernous hemangioma caused hemobilia. Cavernous hemangioma is the most common benign neoplasm of the liver and rarely causes any clinical symptoms or signs, while hemobilia usually occurs secondary to accidental operative or iatrogenic trauma, vascular disease, inflammatory disorders, gallstones, or tumors of the liver. Although invasive or malignant hepatic tumors often result in a communication between the biliary tract and the blood vessels, only one case of hemobilia caused by a benign cavernous hemangioma has ever been reported, but with no details about the patient. Our patient presented to a local hospital with severe melena as the initial main symptom, where ligation of the right hepatic artery was performed. This failed to relieve her symptoms, and she was subsequently referred to our department where a right hepatectomy was performed. Histopathological examination revealed no malignancy combined with the tumor; however, the hemangioma was exposed to the bile duct in segment VIII, which was presumably the cause of the hemobilia. This patient remains in good health almost 6 years after her operation. To the best of our knowledge this is the first case report of hemobilia caused by a cavernous hemangioma, and is accompanied by a detailed analysis.     

Uterine angiomyolipoma associated with pregnancy

Uterine angiomyolipomas are rare lesions composed of mature adipose tissue, smooth muscle, fibrous connective tissue, and blood vessels in varying proportions. We reported the first case of angiomyolipoma associated with a normal pregnancy. Initially, the tumor developed intramurally and could have been confused with a partial molar gestation. After delivery, tumor development was extensive and subserosal, making differential diagnosis from a sarcoma difficult. The question of histological diagnosis, as well as that of immunocytochemical analysis which seems to be helpful in such cases, is discussed here.     

Artificial cells with emphasis on cell encapsulation of genetically engineered cells

Artificial cells are prepared in the laboratory for medical and biotechnological applications. Encapsulated cells are being studied for the treatment of diabetes, liver failure, and other conditions. More recently, there have been extensive studies into the use of encapsulated genetically engineered cells for gene therapy. We recently found that daily orally administered artificial cells, each containing a genetically engineered microorganism, can lower the elevated urea level in uremic rats to normal levels. This may solve the final obstacle of the lack of an effective oral urea removal system for the simple and inexpensive oral treatment of uremia. This is important because 85% of the world's uremic population cannot afford standard dialysis. Other areas of artificial cell application include use in hemoperfusion. Red blood cell substitutes based on modified hemoglobin are already in Phase 3 clinical trials in patients. Artificial cells containing enzymes are being developed for clinical trial in hereditary enzyme deficiency disease and other diseases. They are also being investigated for drug delivery and for use in other applications in biotechnology, chemical engineering, and medicine.     

Mechanical thrombolysis with the Amplatz thrombectomy catheter

The authors describe a rare case of rhinocerebral mucormycosis. This is an acute, suppurative mycosis with poor prognosis. It has a particular affinity for rhinocerebral tissue and less frequently affects pulmonary tissue. Mucormycosis affects immunosuppressed patients and more than 75% of the cases involve patients suffering from acidosis, especially diabetic ketoacidosis. One characteristic feature is that the blood vessels are flooded causing thromboses, infarction and emboli. The disease is spread through the blood vessels or by expansion. The first clinical symptoms can be confused with an early stage of acute sinusitis with mucosanguineous rhinorrhea, facial tumescence and pain. In 50% of the cases there is rhinocerebral and orbital involvement. If the disease remains untreated it can prove fatal in 10 to 14 days. Effective treatment relies on an early diagnosis and prompt administration of intravenous amphotericin B as well as avulsion of the necrotic areas. To date only 200 cases of this severe pathology have been described. The present work is an attempt to throw further light on this disorder.     

PMS after 40: persistence of a stress-related symptom pattern

BACKGROUND AND OBJECTIVE: Various yellow light lasers have been used over the past decade in an attempt to eradicate facial telangiectasia. Based on their power output, spot size, and pulsing characteristics, these lasers belong to one of two categories that exist at either end of a spectrum--high power, short pulse, and large spot size, or low power, long exposure, and small spot size. The copper bromide laser clearly belongs in the latter group, but with higher available power than most other lasers in this group, it exists further along the spectrum toward the region in which the laser parameters might be considered closer to theoretical ideals for treating certain cutaneous vascular pathologies. The objective of this study was to ascertain the role and efficacy of the copper bromide laser on treatment of a variety of facial telangiectasia. STUDY DESIGN/MATERIALS AND METHODS: A total of 570 patients with facial telangiectasia of different diameters and on different regions of the face were treated with the copper bromide laser one or more times and followed up over 5 years. RESULTS: More than 75% clearance was achieved in 70% patients, 50-75% clearance in 17.4% patients, and < 50% clearance in 12.6% patients. Poor results were correlated with anatomical location on the nasal alae and nasal tip and also with vessel size. Very small (< 100 microns) and very large (> 300 microns) vessels did not respond as well as vessels in the 100-300-micron diameter group. Very large vessels responded better to a combination of sclerotherapy and laser treatment. There were no reported long-term adverse effects. CONCLUSION: The copper bromide laser is a safe and effective modality for the treatment of the majority of facial telangiectasia. It is less suited to treating very small vessel lesions such as diffuse erythema, and conversely very large vessels as well as those of the nasal alae. These latter two groups respond better and more permanently to combined sclerotherapy and laser treatment.     

ATM: from gene to function

The identification of ATM , the gene responsible for the pleiotropic recessive disease ataxia telangiectasia, has initiated extensive research to determine the functions of its multifaceted protein product. The ATM protein belongs to a family of protein kinases that share similarities at their C-terminal region with the catalytic domain of phosphatidylinositol 3-kinases. Studies with ataxia telangiectasia (A-T) cells and Atm-deficient mice have shown that ATM is a key regulator of multiple signaling cascades which respond to DNA strand breaks induced by damaging agents or by normal processes, such as meiotic or V(D)J recombination. These responses involve the activation of cell cycle checkpoints, DNA repair and apoptosis. Other roles outside the cell nucleus might be carried out by the cytoplasmic fraction of ATM. In addition, ATM appears to function as a 'caretaker', suppressing tumorigenesis in specific T cell lineages.     

Hibernating myocardium: a hypometabolic state for energy conservation

Because of the anatomical features associated with situs inversus, technical difficulties will be encountered during orthotopic liver transplantation. This report describes the case of a patient with situs inversus totalis and end-stage liver disease from biliary atresia who was treated by segmental orthotopic liver transplantation. The segmental graft was safely placed in the left subphrenic space, and a suitable orientation was obtained for anastomoses of the hilar vessels. Chronic rejection necessitated retransplantation, by the same method, 19 months later. This technique has potential advantages in coping with anatomical obstacles encountered in patients with situs inversus.     

Acute liver necrosis induced by iodine-131-MIBG in the treatment of metastatic carcinoid tumors

Iodine-131-metaiodobenzylguanidine (MIBG) is used in the treatment of carcinoid tumors. Temporary palliation with complete subjective symptomatic response has been reported in these patients. This treatment is usually well tolerated and side-effects are generally limited to nausea, mild hepatic toxicity with spontaneous recovery and temporary myelosuppression. Our case report shows that repeated treatment with [131I]MIBG in a patient with extensive carcinoid liver metastasis may cause severe hepatic toxicity leading to death. Factors such as concomitant use of 5-fluorouracil and the progressive nature of the disease may have contributed to this event.     

Rhizomucor pusillus mucormycosis combined with chlamydiosis in an African grey parrot (Psittacus erithacus erithacus)

This paper describes a case of mucormycosis combined with chlamydiosis in an African grey parrot (Psittacus erithacus erithacus). The clinical signs included diarrhoea, an unsteady gait and a twisted neck. Smears of the spleen, liver and contents of the cloaca stained strongly positive for Chlamydia species. Histologically, hyphae typical of Zygomycetes were observed invading through the walls of blood vessels of the spleen. Rhizomucor pusillus was isolated as a pure culture from the intestines, lungs and liver.     

Expression and localization of angiotensin II type 1 receptor mRNA in rat ocular tissues

OBJECTIVE: Hereditary hemorrhagic telangiectasia (HHT) is an inherited abnormality passed down as a dominant autosomal feature. Recurrent epistaxis usually constitutes the major clinical manifestation of this disease. The unsatisfactory results of conservative therapy have stimulated a research interest for the role of laser photocoagulation in telangiectatic vessels associated with this clinical entity. METHOD: The Nd:YAG laser was used to treat a group of 11 individuals suffering from HHT, all of whom had been previously treated using other modalities. RESULTS AND CONCLUSION: The excellent results of Nd:YAG laser irradiation are addressed in view of all treatment modalities proposed for the treatment of recurrent epistaxis in HHT.     

600 nm flash pumped dye laser for fragile telangiectasia of the elderly

Since 1984, a continuum of controlled studies have been done on the use of the fragile, superficial telangiectasia of the elderly, which were utilized as a model for some of the basic studies of the effects of lasers on the cardiovascular system. We report the use of the 600 nm flash pumped dye laser for the fragile telangiectasia of the elderly in our classification of the Class 2 group. In some vessels studied, the special Teflon-insulated, intravenous electric needle of Kobayashi-Yamada was used. For the bluish vessels, the 600 nm flash pumped dye laser penetrated deeper into the vessel than the 585 nm, but multiple and repeated treatments were needed for clinical effect. Preliminary studies have also shown some initial response in port wine marks and facial telangiectasia.     

Quantitative analysis of the peripheral blood cytotoxic T lymphocyte response in patients with chronic hepatitis C virus infection

Hepatitis C virus (HCV)-specific cytotoxic T lymphocytes (CTL) are present in the peripheral blood and liver of chronically infected patients. The current study was performed to study the relationship between the strength of the CTL response, liver disease severity, and viral load. The results may be summarized as follows: first, using CTL precursor frequency (CTLpf) analysis to quantitate the peripheral blood CTL response, chronically infected patients were less strongly sensitized to a panel of well-defined HCV epitopes than they were to an epitope within the influenza matrix protein. Second, HCV-specific CTLpf did not correlate with disease activity or viral load in the majority of patients on a cross-sectional basis, although it did increase in three patients concomitant with sharp increases in liver disease. Finally, interferon therapy did not enhance the CTLpf against the HCV epitopes studied in these patients, indicating that its antiviral effect is independent of the CTL response. Since the HCV-specific CTLpf in the blood is actually quite low, the CTL may contribute to ongoing liver disease in these patients while being quantitatively inadequate to destroy all of the infected hepatocytes, thereby facilitating HCV persistence and contributing to chronic liver disease.     

Calcium carbonate apatite deposition in the cervical spine with associated vertebral destruction. Case report

This 52-year-old woman developed crystal deposition disease involving the cervical vertebrae. She presented with symptomatic spinal cord compression secondary to extensive calcified lesions in the posterior elements of the cervical spine. Surgical decompression with posterior fusion was performed. Histological examination showed hardened deposits of calcium carbonate involving the soft tissue, and dissolution of the vertebral bone trabeculae. There was no inflammatory response to these deposits. One year postoperatively the patient developed severe pulmonary disease associated with the collagen-vascular disorder, scleroderma (calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia [CREST] syndrome). Calcium carbonate deposition disease represents an unusual clinical entity that is possibly associated with scleroderma or other collagen-vascular diseases, and it is distinct from ligamentum flavum calcification, calcium pyrophosphate deposition disease, and hydroxyapatite deposition disease.     

Hepatic venous compliance and role of liver as a blood reservoir

Changes in hepatic blood volume in response to rapid elevations in hepatic venous pressure were examined in cats using hepatic plethysmography. The liver was intact and received blood from an intact portal vein and hepatic artery. The hepatic blood volume increased in response to elevated venous pressure. Compliance of the hepatic capacitance vessels became greater as the distending venous pressure was increased over the range of venous pressures studied (0-9.4 mmHg). When hepatic venous pressure was elevated to 9.4 MMHg, the hepatic blood volume more than doubled. The liver serves as an important buffer for rapid changes in blood volume, the importance increasing with greater infused volumes of fluid. While overall venous compliance decreased at distending pressures in excess of 5-6 mmHg, the compliance of the hepatic capacitance vessels shows marked increases at pressures above this level. Expansions of the blood volume results in elevations of central venous pressure. Within a few minutes the hepatic capacitance vessels sequester a significant proportion of the added volume. As long as central venous pressure is raised, the liver demonstrates a secondary fluid buffering role by filtering large volumes of fluid across the vascular bed into the peritoneum.