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Neuroretinitis in acquired syphilis.

Syphilitic disease of the retina and/or the optic nerve head, without choroidal involvement, occurred in our 4 cases and in another 19 cases. The condition almost always takes place in the secondary stage, frequently associated with meningitis, and rarely in tertiary meningovascular syphilis. Fluctuating visual loss and floating spots without ocular pain are the presenting symptoms. Retinitis, papillitis, and neuroretinitis are accompanied by an inflammatory reaction in the vitreous and, sometimes, in the aqueous. Paracentral scotomas and blind spot enlargement, related with posterior pole and papillary edema, are the most usual visual field defects. Almost complete visual recovery is the rule in the treated cases, although in some instances cystoid macular edema and retinal ischemia due to endarteritis cause permanent visual loss. Treatment with crystalline penicillin is mandatory in patients with concomitant neurosyphilis, whereas procaine penicillin is seemingly sufficient in those with a normal cerebral spinal fluid examination.

Adult↗

The spectrum of organ and systems pathology in human Lyme disease.

Lymphocytes, plasma cells, and mononuclear phagocytes are frequently found in human tissues infected by the Lyme disease spirochete, Borrelia burgdorferi. Experience has shown that these cells comprise the tissue bed inflammatory infiltrate in Lyme disease affecting the joint synovia, myocardium, and skin. While many differences otherwise exist, Lyme synovitis has lymphoplasmacellular similarities with rheumatoid synovitis, lymphoplasmacellular epimyocarditis similarities with syphilitic myocarditis, and occasionally synovial endarteritis obliterans. Silver staining can demonstrate the spirochete if a careful search is done.

Arthritis, Rheumatoid↗

Skin manifestations of pentazocine abuse.

Seventeen patients who had cutaneous complications following pentazocine injections are presented. The mean age was 50 years; total daily pentazocine dose ranged from 60 to 2,400 mg; evidence of psychiatric illness was present in 94%, and previous drug or alcohol abuse was noted in 65%. Fifty-three percent of our series of patients had a medical or paramedical background. Eighteen percent had diabetes mellitus, and 65% had a personal or family history (or both) of diabetes. A hypothesis is presented for this association. Characteristic histologic findings included fibrosis of the dermis and panniculus, with vascular alterations, fat necrosis with granulomatous inflammation, and vascular thrombosis with occasional endarteritis. We emphasize that medical and paramedical personnel and patients with a personal or family history of diabetes should be added to the group of patients considered to be at special risk for cutaneous complications of pentazocine injections.

Adult↗

Lipomembranous changes in chronic panniculitis.

The clinical and histopathologic findings in 13 patients with lipomembranous changes in the subcutaneous adipose tissue as part of the inflammatory reaction are presented. Nine patients had clinical evidence of vascular disease and four had clinical evidence of connective tissue disease. Histopathologic evidence of endarteritis obliterans, venous stasis, and hemorrhage was present in more than half the patients, and the clinical lesion of liposclerosis was frequently present. These findings suggest that the histologic changes of lipomembranous panniculitis may be the result of an inflammatory reaction in patients who have the liposclerosis of venous insufficiency with connective tissue disease or previous leg ischemia or both.

Adipose Tissue↗

Radiation accidents.

It is essential that emergency physicians understand ways to manage patients contaminated by radioactive materials and/or exposed to external radiation sources. Contamination accidents require careful surveys to identify the metabolic pathway of the radionuclides to guide prognosis and treatment. The level of treatment required will depend on careful surveys and meticulous decontamination. There is no specific therapy for the acute radiation syndrome. Prophylactic antibodies are desirable. For severely exposed patients treatment is similar to the supportive care given to patients undergoing organ transplantation. For high-dose extremity injury, no methods have been developed to reverse the fibrosing endarteritis that eventually leads to tissue death so frequently found with this type of injury. Although the Three Mile Island episode of March 1979 created tremendous public concern, there were no radiation injuries. The contamination outside the reactor building and the release of radioiodine were negligible. The accidental fuel element meltdown at Chernobyl, USSR, resulted in many cases of acute radiation syndrome. More than 100,000 people were exposed to high levels of radioactive fallout. The general principles outlined here are applicable to accidents of that degree of severity.

Bone Marrow Transplantation↗

[Sneddon's syndrome: review of the literature apropos of a case].

The authors report the case of a young woman with Sneddon's syndrome and expose literature review. This rare entity is characterized by idiopathic livedo reticularis, ischemic stroke, and occasionally, mild arterial hypertension. Skin biopsy shows endarteritis obliterans of deep dermal arteries. The pathogenesis of this disorder is still unknown and the treatment is not clearly established.

Adult↗

Physiological and pathological morphology of the umbilical and placental circulation.

Closure mechanisms of fetal vessels are essential after birth. They are physiological. The speedy and characteristic constriction is made possible by the special construction of the fetal vessels. The so-called "folds of Hoboken" in the umbilical artery initially form tapered constrictions which quickly extend to longer sections of the vessel. The contractions continue to include the chorion plate and the villi. Due to a lack of lamina elastica interna, a protrusion of pear shaped, expanded mediamyocytes and myofibroblasts is possible which reduces the cross-sectional area of these vessels. Similar, but lasting reductions in the cross-section of the villous arteries due to a fibrous-muscular media occur in the second half of pregnancy (IVth sign of maturity). Closure of the fetal vessels during intrauterine life leads to placental insufficiency of fetal death in utero. The endarteritis obliterans closes villous vessels by means of a connective tissue plug which starts on one side of the vessel and continues across the whole width of the lumen. The periphery dependent of these villi remains avascular, as the placenta is limited in its resorptive capabilities. In the case of intrauterine asphyxia, intravasal fibrinthrombi form as a sign of subacute insufficiency in the utero-placental circulation. A decompression collapse occurs in the fetal circulation, when fetal death occurs with continuing maternal circulation.

Female↗

Hashimoto's thyroiditis associated with Riedel's thyroiditis and retroperitoneal fibrosis.

This article describe the case of a 38-year-old woman with simultaneous involvement of the thyroid by Hashimoto's thyroiditis and Riedels's disease, associated with retroperitoneal fibrosis and lipidic endarteritis. According to a large review of the literature on the occurrence of this rare condition the difficulty in sharply defining the two thyroid processes is discussed. The chronology of the events is analyzed, and etiopathogenic hypotheses are listed with emphasis on the relationship between the vascular lesions and the onset of the fibrosing process.

Adult↗

Long-term (4- to 6-year) outcome of Palmaz-Schatz stenting: paucity of late clinical stent-related problems.

OBJECTIVES: The purpose of this prospective single-center study was to evaluate the longer-term outcome of Palmaz-Schatz stenting in the treatment of native coronary and saphenous vein bypass graft disease. BACKGROUND: The STRESS (Stent Restenosis Study) and BENESTENT (Belgian Netherlands Stent) trials have demonstrated a decrease in both angiographic restenosis and the need for repeat revascularization in the 1st year for vessels treated by stenting rather than balloon angioplasty. Longer-term (1 to 5 years) clinical results of Palmaz-Schatz stenting are not yet well established. Late migration of the stent, metal fatigue, endarteritis and late restenosis have all been proposed as potential late clinical complications of coronary stent implantation. METHODS: The study cohort consisted of 175 consecutive patients who underwent elective placement of 194 Palmaz-Schatz stents in 185 vessels. Clinical events (death, myocardial infarction, recurrent angina or any revascularization) were assessed at 6 weeks, 2, 4 and 6 months, 1 year and yearly thereafter. Clinical follow-up was available on all patients at a mean +/- SD of 54 +/- 17 months. RESULTS: Angiographic success was achieved in 173 patients (98.9%); angiographic restenosis was observed at 6 months in 26.1% of target sites. The survival rate was 86.7% at 5 years, with a 5-year event-free survival rate decreasing progressively to 50.7%, reflecting primarily repeat revascularization procedures (41.2% at 5 years). However, the rate of repeat revascularization of the treatment site (target site revascularization [TSR]) was 14.4%, 17.7% and 19.8% at 1, 3 and 5 years, respectively, with late (> 1 year) TSR driven by in-stent restenosis in only 3 patients (1.7%). Rates of both 5-year survival (70.5% vs. 93.4%) and event-free survival (21.1% vs. 63.3%) were lower for patients who underwent saphenous vein graft (SVG) stenting than for those with native coronary artery stenting. However, 5-year TSR rates were similar for SVGs (21.9%) and native vessels (19.2%), indicating that the higher incidence of repeat revascularization for SVGs was due to an increase in non-TSR, driven by progressive disease at other sites. CONCLUSIONS: The long-term outcome of stenting shows stability of the treated lesion, with only a slight increase in TSR between 2 and 5 years (17.1% to 19.8%). The progressive increase in repeat revascularization over that period (24% to 41%) and most ongoing late events can be attributed to the progression of coronary disease at other sites, rather than to late deterioration of the stent result itself. Such non-TSR events account for the majority of clinical events in the patients who underwent SVG stenting.

Aged↗

Buerger's disease in the 21st century: diagnosis, clinical features, and therapy.

Buerger's disease (thromboangiitis obliterans or TAO) is a clinical syndrome characterized by the development of segmental thrombotic occlusions of the medium and small arteries of the extremities. It is clinically and pathologically distinguishable from arteriosclerosis and necrotizing arteritis. Afflicted patients are mostly young, male, inveterate tobacco smokers who present with distal extremity ischemia, ischemic ulcers, or frank gangrene of the toes or fingers. Large arteries are typically spared, as are the coronary, cerebral, and visceral circulations. While mortality is not increased, patients with Buerger's disease often suffer from severe ischemic pain and tissue loss culminating in minor and major limb amputation. Clinical diagnostic criteria generally include the following: (1) history of smoking or tobacco abuse; (2) age of onset less than 45 to 50 years; (3) infrapopliteal, segmental arterial occlusions with sparing of the proximal vasculature; (4) frequent distal upper extremity arterial involvement (Raynaud's syndrome or digital ulceration); (5) superficial phlebitis; and (6) exclusion of arteriosclerosis, diabetes, true arteritis, proximal embolic source, and hypercoagulable states. Typical arteriographic patterns have been described that are suggestive, but not pathognomonic. While the cause of Buerger's disease remains unknown, the disease onset and clinical course are inextricably linked to tobacco abuse. Acute Buerger's disease is characterized histopathologically by intensely cellular vessel wall inflammation, giant cell foci, and hypercellular thrombi, but with preservation of the elastic lamina and the overall vascular wall architecture. Most investigators feel that Buerger's disease is an immune-mediated endarteritis; recent immunocytochemical studies have identified the linear deposition of immunoglobulins and complement factors along the elastic lamina. The inciting antigen has not been discovered. Tobacco abstinence generally results in disease quiescence and remains the mainstay of treatment.

Humans↗

Cholesterol crystal embolism: diagnosis and treatment.

Cholesterol crystal embolization (CCE) is a dreaded complication of radiology, vascular surgery, and/or anticoagulation in patients with atherosclerosis and ulcerated aortic plaques. It also represents a cause of early graft failure and of poor results of renal artery surgery. Crystals lodge in small caliber renal arteries, where they induce early, transitory thrombosis followed by delayed, definitive obstruction by endarteritis, accompanied by evidence of inflammation and eosinophilia. Massive CCE leads to early oligoanuria. In subacute forms, renal insufficiency is often delayed by weeks or months following the triggering event. A third, chronic subset of CCE is easily mistaken for atherosclerotic renal ischemia and/or nephrosclerosis. The kidney is rarely the sole organ involved in acute/subacute forms, in which the central nervous system, the coronary arteries, the spinal cord, and the mesenteric and pancreatic blood supply compromise represent the main causes of death. Cutaneous, retinal, and muscle involvement allow diagnosis by inspection or scarcely invasive biopsies in about 80% of cases, whereas renal biopsy as the only diagnostic procedure is required in 20% of cases. Prevention is based on avoidance of endovascular radiology maneuvers, vascular surgery, and excess anticoagulation in atherosclerotic patients. Treatment of acute/subacute forms of renal insufficiency consisting of stopping anticoagulation and forbidding any new radiologic and/or vascular surgery procedure; treating hypertension with angiotensin 2 antagonists and vasodilators, strict volemic control by loop diuretics and ultrafiltration, along with parenteral nutrition and prednisone, has been credited with improved outcome. Iloprost may obtain favorable results. Statins definitely ameliorate the renal and patient's prognosis.

Diagnosis, Differential↗

Necrotizing gangrene of the genitalia and perineum.

BACKGROUND: Necrotizing gangrene of the genitalia and perineum is a fulminant, life-threatening condition. This infection is usually polymicrobial and may be idiopathic or secondary to local trauma or surgery. Histologically, it is characterized by obliterative endarteritis and thrombosis of the subcutaneous vessels, fascial necrosis, and leukocytic infiltration. Mortality rates of 25-75% have been reported. Most cases of necrotizing gangrene begin insidiously, with scrotal discomfort and malaise. Later, erythema, increasing pain, and swelling, associated with fever and chills, develop. A biopsy is useful to confirm the clinical diagnosis and to obtain culture samples. Ultrasound imaging may reveal gas or testicular involvement and may help to distinguish this infection from other causes of scrotal pathology. MATERIALS AND METHODS: Fifteen patients with necrotizing gangrene of the genitalia and perineum, seen at the Dermatology and Plastic Surgery Sections of our Institutions between 1994 and 1999, are described. RESULTS: This series included 11 men (73%) and four women (27%), aged 39-68 years (mean, 51 years). In our series, Clostridium perfingens, Staphylococcus aureus, Proteus mirabilis, Pseudomonas aeruginosa, Streptococcus viridans, Acinetobacter baumani, Escherichia coli, and Candida albicans were isolated. Hemodynamic stabilization and monitoring were performed in all patients. Intravenous antimicrobial therapy was promptly instituted. In most cases, two or more drugs were used. Concurrent surgical debridement of all necrotic areas was always required. When needed, split-thickness skin grafts were used to cover the penile shaft. Expanded mesh grafts were used to reconstruct the vulva and other denuded beds. The survival rate in this series was 87%. CONCLUSIONS: Necrotizing gangrene of the genitalia and perineum continues to be a diagnostic and therapeutic challenge. The usual polymicrobial infection with vascular involvement demands hemodynamic stabilization, systemic antimicrobial therapy, and surgical debridement. In some patients, genital, perineal, and abdominal wall reconstruction is also required.

Adult↗

Intragraft events preceding chronic renal allograft rejection in a modified tolerance protocol.

BACKGROUND: Inbred miniature swine treated for 12 days with high-dose cyclosporine A develop tolerance to histocompatibility complex (MHC) class I-mismatched renal allografts. When this protocol was modified by adding thymectomy before transplant, all animals developed acute rejection. Thereafter, by day 100, one half developed chronic rejection (progression group) and the other half recovered (recovery group). This provides an excellent experimental model to identify the mechanisms of chronic rejection as well as the early changes that may predict chronic rejection. METHODS: We assessed the cellular infiltration, immune activation, humoral immunity, and cell- and antibody-mediated graft injury in the progression and the recovery groups. In addition, we also examined circulating donor reactive cytotoxic T lymphocyte (CTL) and antidonor antibody in both groups. RESULTS: From days 8 to 18 after transplantation, the two groups were indistinguishable. Both showed acute rejection with endarteritis (type II); had IgG and IgM deposition in glomeruli and small vessels; had an infiltrate with similar numbers of T cells, proliferating (PCNA+) and activated (interleukin-2 receptor+) cells; and had a similar degree of parenchymal cell apoptosis [in situ DNA nick-end labeling (TUNEL)+]. However, by days 30 to 60, the two groups could be distinguished by several intragraft features. The recovery group became tolerant and had diminished T-cell infiltration, activation and proliferation, and no detectable antibody deposition. The number of TUNEL+-injured parenchymal cells decreased. In contrast, the progression group showed persistent cell infiltration with activation and proliferation. Significantly prominent TUNEL+ apoptotic parenchymal cells in tubules, glomeruli, peritubular capillaries and arteries were seen from day 30 to day 100. Circulating donor reactive CTL and antidonor class I IgG were detected in the progression group at higher levels than in the recovery group from days 30 to 60. CONCLUSION: In tolerance-induction protocols, unstable tolerance induction is associated with the persistent immunologic activation that mediates immunologic destruction of graft parenchymal cells and chronic rejection. Certain of the described immunopathologic findings (activation, proliferation, apoptosis, and antibody deposition) may be useful in distinguishing the type of rejection, that is, whether the allograft will progress to chronic rejection or recovery.

Acute Disease↗

Injection therapy for endoscopic haemostasis.

Endoscopic injection is widely used for the arrest of active ulcer bleeding and for prevention of re-bleeding from ulcers with visible vessels. Although of proven value in clinical trials, mechanisms of action are unclear; tamponade, vasoconstriction, endarteritis and a direct effect upon the clotting process at the site of the arterial defect have been proposed. Clinical trials show that dilute adrenaline is an effective agent and that the addition of sclerosants or alcohol confirms no extra benefit, yet risks serious side-effects. The best results are associated with injection of fibrin glue or thrombin which stimulate formation of a stable blood clot. The efficacy of injection, thermal modalities such as the heater probe and electrocoagulation using BICAP are comparable. In general, there is an advantage in combining injection with a thermal modality, although this may have merit in patients with severe, active ulcer bleeding. Patients who re-bleed following successful primary haemostatic injection treatment can be considered for further endoscopic intervention, but the decision to undertake a surgical operation or repeat endoscopic therapy is a matter of clinical judgement.

Gastrointestinal Hemorrhage↗

Treatment of coarctation and late complications in the adult.

This article discusses coarctation of the aorta in the adult. Effective treatments for coarctation have come from surgery since 1945 and from interventional cardiology since 1982. Long-term outcome data are available only for surgical approaches. Thirty-year survival rate is 72% to 82%. Complications include recoarctation or residual coarctation, hypertension, aneurysms at the repair site, spinal cord injury. Other sequelae include bicuspid aortic valve disease, ascending aortic aneurysm, premature coronary disease, and infective endocarditis or endarteritis. Interventional catheter therapy is now the preferred therapy for recurrent coarctation, when the anatomy permits and necessary skills are available. Its use in native or unoperated coarctation is less well established. Treatment may be with balloon angioplasty alone or with a stent. Outcomes are good in skilled hands, but residual or recurrent coarctation with resultant hypertension and repair site aneurysms can occur. Catheter treatment can cause death from aortic rupture and dissection, but mortality compares favorably with surgery if coarctation is recurrent, and perhaps for initial treatment.

Adult↗

Absence of ischemia in telangiectasias of chronic radiation proctopathy.

BACKGROUND AND STUDY AIMS: It has been postulated that chronic radiation proctopathy, clinically manifested by hematochezia and by the appearance of multiple telangiectasias, is caused by ischemia. This theory is based on reports that appeared in the 1980s which described obliterative endarteritis in patients with chronic radiation-induced ulcers. However, bleeding from radiation proctopathy is typically successfully treated endoscopically by widespread tissue coagulation, and the complications that would be expected to occur if the tissue was ischemic, such as poor wound healing, generally do not arise. We therefore hypothesized that the ischemia theory is incorrect and that rectal capillary oxygen saturation is normal in patients with telangiectasias of chronic radiation proctopathy. PATIENTS AND METHODS: We developed a visible-light spectroscopy device that measures mucosal capillary hemoglobin oxygen saturation during endoscopy (having reported its operating characteristics previously). We prospectively studied 20 patients who had typical findings of multiple rectal telangiectasias, 1 - 20 years after undergoing external-beam irradiation for prostate or rectal carcinoma. We measured and compared the mucosal capillary oxygen saturations in the affected areas of the distal rectum and in endoscopically normal areas in the rectosigmoid colon. RESULTS: Mucosal oxygenation was normal in all 20 patients in affected areas (64 % - 80 %) and in unaffected areas (63 % - 75 %). The mean mucosal hemoglobin oxygen saturation was actually slightly higher in the affected areas of the rectum than in the uninvolved rectosigmoid colon (73 % vs. 69 %, P < 0.01). CONCLUSIONS: The common form of chronic radiation proctopathy, characterized by multiple telangiectasias without ulcers or strictures, is not associated with ongoing mucosal ischemia. This finding may explain why endoscopic treatment of this disorder, in which large areas of the mucosa are coagulated with argon plasma or other treatment modalities that cause widespread ulceration, does not typically result in complications from poor wound healing.

Aged↗

[Post-actinic retrobulbar optic neuropathy].

BACKGROUND: Radiation optic neuropathy (RON) is a rare, unpredictable, late complication of radiotherapy secondary to obliterative endarteritis. Tumor recurrence has to be ruled out by a clinical and neuroradiological examination. METHODS: Five patients with RON were investigated by magnetic resonance imaging (MRI) during 1992. RESULTS: Radiation-induced lesions of the intracranial visual pathways were easily visible on MRI. Without Gadolinium, a sectorial swelling was detectable, which markedly enhanced with Gadolinium. Intracranial optic nerve was affected in 5/5 cases, optic chiasm in 3/5 cases, and optic tract in 2/5 cases. CONCLUSIONS: MRI is the examination of choice when RON is suspected: it will easily delineate the extent of the lesion, and compression/infiltration by a recurrent tumor will be formally ruled out. A segmental swelling of visual pathway with marked Gadolinium enhancement on MRI is highly suggestive of radionecrosis.

Cranial Irradiation↗

Intraarterial drug abuse: new treatment options.

Accidental, intraarterial injections of abused substances continue to be difficult management problems because of severe vascular complications. Vasospasm probably plays an important role in the pathophysiology of this injury, in addition to the more accepted roles of thrombosis and endarteritis. Three patients with severe, upper extremity ischemia after intraarterial injection of heroin, methamphetamine, and meperidine were treated with intraarterial tolazoline and streptokinase, along with intravenous heparin. All three patients had improved perfusion, assessed both clinically and by angiography. The responses to treatment in these patients demonstrated the roles of vasospasm and thrombosis in the origin of this injury and the therapeutic benefits of appropriately administered vasodilators and thrombolytic agents.

Adult↗