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Varicella-zoster virus DNA in CSF and arteries in delayed contralateral hemiplegia: evidence for viral invasion of cerebral arteries.

A 78-year-old woman presented with a right basal ganglia infarct 6 weeks after a left herpes zoster ophthalmicus. MR angiography showed focal segmental stenosis of the proximal segments of the anterior, middle, and posterior cerebral arteries. Varicella DNA was detected in the CSF by polymerase chain reaction (PCR). Treated with dexamethasone and acyclovir without improvement, she died 1 month later. There was focal endarteritis in the left anterior, middle, and posterior cerebral arteries at autopsy. Varicella DNA was detected by PCR of extracts from these vessels but not from the arteries on the right side. This study provides further evidence that the vasculopathy after herpes zoster ophthalmicus results from direct viral invasion of the vessel wall.

Aged↗

Pulmonary dirofilariasis in a Japanese hare, Lepus brachyurus angustidens.

A case of pulmonary dirofilariasis in a Japanese hare, Lepus brachyurus angustidens captured in Aomori Prefecture, was first reported in Japan. Two dead worms recovered from the pulmonary artery and detected within an organized arterial thrombus in the lung, were identified as a male and a female young adult Dirofilaria immitis, respectively. Principal lesions in the lung were associated with pulmonary arterial degeneration and consisted of rugose and villous intimal fibrosis, endarteritis with infiltration by numerous eosinophils and mononuclear inflammatory cells, and thrombosis.

Animals↗

Cardiac abscess following percutaneous transluminal coronary angioplasty.

We report the first case of myocardial abscess directly related to percutaneous transluminal angioplasty (PTCA). Infectious complications of PTCA are very rare and limited to a few cases of groin infection and septic endarteritis most often after repeated procedures. In our patient, a problematic and repeated procedure probably led to a direct colonization and subsequent infection of an intimal dissection of the right coronary artery. Standard aseptic techniques can be inadequate in the case of early repuncture or if there is an indwelling line. Prophylactic antibiotic treatment should be considered in this case, although its usefulness has not been yet formally demonstrated.

Abscess↗

Human pulmonary dirofilariasis: analysis of 24 cases from São Paulo, Brazil.

STUDY OBJECTIVE: To present the clinical, radiologic, and pathologic aspects of 24 cases of human pulmonary dirofilariasis (HPD) from São Paulo, Brazil. DESIGN: Retrospective study of 24 patients with a confirmed diagnosis of HPD over a 14-year period (from February 1982 to June 1996). SETTING: Thoracic Surgery and Pulmonary Division, University of São Paulo and Hospital Albert Einstein, São Paulo, Brazil. RESULTS: Seventeen patients were male (70.1%) and seven were female (29.9%). Their mean age was 51.4 years. Fifty-four percent of the patients were asymptomatic and 75% had a well-circumscribed noncalcified peripheral subpleural pulmonary nodule on the chest radiograph and thoracic CT scan, located preferentially in the lower lobes. The diagnosis was made after thoracotomy and wedge resections in 16 patients, by videothoracoscopy in six, after a pleural biopsy in one, and after necropsy in one. The pathologic examination of all the nodules revealed a central zone of necrosis, surrounded by a narrow granulomatous zone and peripherally by fibrous tissue. Pulmonary vessels exhibit varying degrees of endarteritis. In all cases, a dead worm, usually necrotic and fragmented, was found. CONCLUSIONS: A subpleural, noncalcified pulmonary nodule in the appropriate clinical and epidemiologic setting should alert the clinician, radiologist, or pathologist to the possibility of Dirofilaria. HPD should be considered in the differential diagnosis of pulmonary nodules.

Adolescent↗

Congenital syphilis: unique clinical presentation in three preterm newborns.

Three preterm newborns affected by congenital syphilis, born to mothers not adequately treated during pregnancy, are described. The clinical picture is characterized by a severe cholestatic hepatopathy and, in the two surviving patients, by an unusually wide ischemic-hemorrhagic lesion of the cerebral parenchyma. This lesion is probably due to a syphilitic endarteritis, and has rarely been described before in preterm infants.

Brain Ischemia↗

Percutaneous closure of ductus arteriosus with the amplatzer prosthesis. The Brazilian experience.

OBJECTIVE - To report the results of percutaneous occlusion of persistent ductus arteriosus with the Amplatzer prosthesis in 2 Brazilian cardiological centers. METHODS - From May 1998 to July 2000, 33 patients with clinical and laboratory diagnosis of persistent ductus arteriosus underwent attempts at percutaneous implantation of the Amplatzer prosthesis. The median age was 36 months (from 6 months to 38 years), and the median weight was 14kg (from 6 to 92kg). Sixteen patients (48.5%) were under 2 years of age at the time of the procedure. All patients were followed up with periodical clinical and echocardiographic evaluations to assess the presence and degree of residual shunt and possible complications, such as pseudocoarctation of the aorta and left pulmonary artery stenosis. RESULTS - The minimum diameter of the arterial ducts ranged from 2.5 to 7.0mm (mean of 4.0+/-1.0, median of 3.9). The rate of success for implantation of the prosthesis was 100%. Femoral pulse was lost in 1 patient. The echocardiogram revealed total closure prior to hospital discharge in 30 patients, and in the follow-up visit 3 months later in the 3 remaining patients. The mean follow-up duration was 6.4+/-3.4 months. All patients were clinically well, asymptomatic, and did not need medication. No patient had narrowing of the left pulmonary artery or of the aorta. No early or late embolic events occurred, nor did infectious endarteritis. A new hospital admission was not required for any patient. CONCLUSION - The Amplatzer prosthesis for persistent ductus arteriosus is safe and highly effective for occlusion of ductus arteriosus of varied diameters, including large ones in small symptomatic infants.

Adolescent↗

Acute humoral rejection in kidney transplantation: II. Morphology, immunopathology, and pathologic classification.

The incidence of acute humoral rejection (AHR) in renal allograft biopsies has been difficult to determine because widely accepted diagnostic criteria have not been established. C4d deposition in peritubular capillaries (PTC) of renal allografts has been proposed as a useful marker for AHR. This study was designed to test the relative value of C4d staining, histology, and serology in the diagnosis of AHR. Of 232 consecutive kidney transplants performed at a single institution from July 1995 to July 1999, all patients (n = 67) who developed acute rejection within the first 3 mo and had a renal biopsy with available frozen tissue at acute rejection onset, as well as posttransplant sera within 30 d of the biopsy, were included in this study. Hematoxylin and eosin and periodic acid-Schiff stained sections were scored for glomerular, vascular, and tubulointerstitial pathology. C4d staining of cryostat sections was done by a sensitive three-layer immunofluorescence method. Donor-specific antibodies (DSA) were detected in posttransplant recipient sera using antihuman-globulin-enhanced T cell and B cell cytotoxicity assays and/or flow cytometry. Widespread C4d staining in PTC was present in 30% (20 of 67) of all acute rejection biopsies. The initial histologic diagnoses of the C4d(+) acute rejection cases were as follows: AHR only, 30%; acute cellular rejection (ACR) and AHR, 45%; ACR (CCTT types 1 or 2) alone, 15%; and acute tubular injury (ATI), 10%. The distinguishing morphologic features in C4d(+) versus C4d(-) acute rejection cases included the following: neutrophils in PTC, 65% versus 9%; neutrophilic glomerulitis, 55% versus 4%; neutrophilic tubulitis, 55% versus 9%; severe ATI, 75% versus 9%; and fibrinoid necrosis in glomeruli, 20% versus 0%, or arteries, 25% versus 0%; all P < 0.01. Mononuclear cell tubulitis was more common in the C4d(-) group (70% versus 100%; P < 0.01). No significant difference between C4d(+) and C4d(-) acute rejection was noted for endarteritis, 25% versus 32%; interstitial inflammation (mean % cortex), 27.2 +/- 27% versus 38 +/- 21%; interstitial hemorrhage, 25% versus 15%; or infarcts, 5% versus 2%. DSA were present in 90% (18 of 20) of the C4d(+) cases compared with 2% (1 of 47) in the C4d(-) acute rejection cases (P < 0.001). The pathology of the C4d(+) but DSA(-) cases was not distinguishable from the C4d(+), DSA(+) cases. The C4d(+) DSA(-) cases may be due to non-HLA antibodies or subthreshold levels of DSA. The sensitivity of C4d staining is 95% in the diagnosis of AHR compared with the donor-specific antibody test (90%). Overall, eight grafts were lost to acute rejection in the first year, of which 75% (6 of 8) had AHR. The 1-yr graft failure rate was 27% (4 of 15) for those AHR cases with only capillary neutrophils versus 40% (2 of 5) for those who also had fibrinoid necrosis of arteries. In comparison, the 1-yr graft failure rates were 3% and 7%, respectively, in ACR 1 (Banff/CCTT type 1) and ACR 2 (Banff/CCTT type 2) C4d(-) groups. A substantial fraction (30%) of biopsy-confirmed acute rejection episodes have a component of AHR as judged by C4d staining; most (90%), but not all, have detectable DSA. AHR may be overlooked in the presence of ACR or ATI by histology or negative serology, arguing for routine C4d staining of renal allograft biopsies. Because AHR has a distinct therapy and prognosis, we propose that it should be classified separately from ACR, with further sub-classification into AHR 1 (neutrophilic capillary involvement) and AHR 2 (arterial fibrinoid necrosis).

Acute Disease↗

Persistent ductus arteriosus in the adult: clinical features and experience with percutaneous closure.

Persistent ductus arteriosus in the adult carries a risk for becoming symptomatic with dyspnoea or palpitations, developing infective endarteritis, and finally for developing pulmonary hypertension with ensuing heart failure morbidity and mortality. Surgical closure is considered more and more to be outdated as first choice treatment since percutaneous closure is a safe and effective alternative. Only the Eisenmenger syndrome remains a clear contra-indication for closure, whether surgical or interventional. We report on our own experience in nine patients with persistent ductus arteriosus including eight cases who underwent percutaneous closure and one patient with Eisenmenger syndrome. In addition, our experience with clinical features and therapeutic modalities is related to the literature.

Adolescent↗

Nematode larvae (Spirurida: Physalopteridae) causing infarction of the bowel in an infant.

A case is reported of an 11-month-old baby suffering from gangrene of the distal portion of the small bowel due to infestation by larval worms belonging to the order Spirurida, family Physalopteridae and probably the genus Physaloptera. Microscopically, the resected bowel showed ischaemic necrosis, eosinophilic granulomata and larval worms in the lumen and wall of blood vessels. The inflammatory process extended into the mesentery, where the vessels showed focal necroses, thrombosis and endarteritis. Infection in this child in the Queensland countryside is considered to have resulted from ingestion of insects on grass eaten when at play, the grass having been contaminated by bandicoots.

Humans↗

Mycotic pseudoaneurysm associated with aortic coarctation.

Development of a mycotic aneurysm or pseudoaneurysm after subacute bacterial endarteritis is uncommon. Nonetheless, patients with coarctation of the aorta are more likely to develop this complication. We describe a case of a large pseudoaneurysm discovered in a child with a previously undiagnosed aorta coarctation. Successful repair was performed with the aid of partial left heart bypass and the use of an interposition graft. A high index of suspicion is necessary to accurately diagnose this rare but life-threatening entity.

Aneurysm, False↗

Recurrent artery of Heubner: Otto Heubner's description of the artery and his influence on pediatrics in Germany.

Although the recurrent artery of Heubner is one of the best known cerebral arteries, little has been written in the neurosurgical or anatomical literature about its discovery. The artery is of primary importance to cerebrovascular surgeons, who identify it during clipping of anterior communicating artery aneurysms. Johann Otto Leonhardt Heubner (1843-1926), who described this artery in 1872, is better known as the father of German pediatrics. He was appointed to the first professorship in Germany exclusively devoted to pediatrics at the Charité Children's Clinic of Berlin University. Although he initially studied internal medicine in Leipzig under Carl Reinhold August Wunderlich and Ernst Leberecht Wagner, his early research involved anatomical studies of the circulation of the brain, from which he described syphilitic endarteritis (Heubner's disease). Finding morphological studies inconclusive, he turned to more physiological experiments. Together with the physiologist Max Rubner, Heubner performed important studies on energy metabolism in infancy, creating the notion of the nutrition quotient. In this article the authors review Heubner's life and scientific discoveries.

Anterior Cerebral Artery↗

Rapidly progressive dementia due to Mycobacterium neoaurum meningoencephalitis.

Dementia developed in a patient with widespread neurologic manifestations; she died within 5 months. Pathologic findings showed granulomatous inflammation with caseation necrosis, foreign body-type giant cells, and proliferative endarteritis with vascular occlusions. Broad-range polymerase chain reaction identified Mycobacterium neoaurum as the possible pathogen. Central nervous system infection by M. neoaurum may result in rapidly progressive dementia.

Dementia↗

A case of primary syphilis in the rectum.

A 30-yr-old man was referred for suspicious rectal cancer because of ulcerated lesions in the rectum and a palpable mass in left inguinal area. Sigmoidoscopy showed two indurated masses and histologic evaluation of biopsy revealed obliterative endarteritis with heavy plasma cell infiltration. Both venereal disease research laboratories (VDRL) and fluorescent treponemal antibody absorption (FTA-ABS) tests were positive. After injection of penicillin G benzathine for 3 weeks, the rectal chancre and the palpable mass disappeared.

Adult↗

Postmortem studies of the heart in three fatal cases of the eosinophilia-myalgia syndrome.

OBJECTIVE: To examine the hearts of individuals who died from the eosinophilia-myalgia syndrome associated with ingestion of L-tryptophan, with particular attention paid to the coronary arteries, the neural structures, and the conduction system of the heart because of reported terminal disturbances of cardiac rhythm and conduction. STUDY MATERIAL: Three hearts fixed in neutral formalin and well preserved with all the relevant areas of conduction system intact. METHODS: Light microscopic examination of subserial sections of the sinus node, atrioventricular node and His bundle, coronary chemoreceptor and regional nerves, ganglia, and small coronary arteries. Routine stains used were Goldner trichrome and Verhoeff-van Gieson. RESULTS: Arterial abnormalities were numerous and primarily of two types: focal fibromuscular dysplasia causing moderate to severe narrowing, as well as endarteritis and panarteritis. Extensive examples of neuritis and ganglionitis were present throughout the heart, including the conduction system, where arterial abnormalities were also abundant. In the coronary chemoreceptor there were both old and new lesions comprising focal inflammation with degeneration as well as older areas of fibrotic destruction. Within the sinus node, areas of dense fibrosis replaced all nodal tissue. These abnormalities were similar in nature and extent in all three hearts. CONCLUSIONS: The pathologic lesions present in the coronary arteries, neural structures, and conduction system of the heart in patients who died from the eosinophilia-myalgia syndrome provide a suitable anatomic substrate for substantial cardiac electrical instability, including the occurrence of sudden death. In cases of unexplained cardiac electrical instability or sudden unexpected death an inquiry should be made about previous use of L-tryptophan. In patients with the eosinophilia-myalgia syndrome, the possibility of cardiac electrical instability should be considered as part of long-range clinical management.

Aged↗

Nonvalvular infections of the cardiovascular system.

PURPOSE: To review the risk factors, clinical presentation, diagnosis, and treatment of nonvalvular infections of the cardiovascular system. DATA SOURCES: A MEDLINE search of English-language articles from 1966 to 1993 relating to myocardial abscesses, mural endocarditis, infective endarteritis and mycotic aneurysms, infection of pacemakers and implantable defibrillators, prosthetic vascular graft infections, and infected atrial myxomas; manual review of article bibliographies. STUDY SELECTION AND DATA EXTRACTION: Case series and single reports, prospective and retrospective clinical studies, autopsy studies, and reviews were selected if they contained sufficient information about the prevalence, clinical manifestations, microbiologic features, management, and outcome of nonvalvular cardiovascular infections. RESULTS: Nonvalvular infections of the cardiovascular system most commonly occur on previously damaged endocardium or vascular intima and are usually associated with intravascular devices such as graft material or pacemakers. Rarely, they can involve primary cardiac tumors such as myxomas. Most patients affected are beyond the fifth decade of life. Risk factors include cardiovascular disease, diabetes mellitus, and malignancy. The sexes are affected equally. The clinical presentations of nonvalvular endovascular infections are subtle and diagnoses are difficult to make, often requiring not only a high index of suspicion but also the use of sophisticated radiologic techniques. Hence, for many of these infections, the diagnosis is made late in the course of the infection, and survival rates are poor. Complications include peripheral embolization, cardiac rupture, vascular aneurysm rupture, and pericarditis. Therapy frequently involves surgical intervention in addition to the use of antibiotics. CONCLUSIONS: Although uncommon, nonvalvular infections of the cardiovascular system will increase in frequency as the use of implantable devices and prosthetic materials increases in the elderly. Studies are needed to determine the most appropriate diagnostic methods, treatment regimens, and methods for prevention of these infections.

Cardiovascular Diseases↗

HLA-B27-associated cardiac disease.

PURPOSE: To review the available information about cardiac disease in relation to the immunogenetic marker HLA-B27 and the inflammatory disorders associated with it (seronegative spondyloarthropathies). DATA SOURCES: English-language clinical studies, case reports, and reviews published in 1995 or earlier were identified from a search of the MEDLINE database. These papers, textbooks, and other bibliographic sources were used to identify other relevant publications, enabling collection of references from 1936 through 1995 in a stepwise manner. STUDY SELECTION: Clinical studies, case reports, and topical reviews on the HLA-B27 histocompatibility locus, seronegative spondyloarthropathies, and cardiac disease were selected. DATA EXTRACTION: Information from systemic or hypothesis-driven studies, including those showing clinically and statistically meaningful associations between disorders, and additional relevant information from case reports and reviews is presented. DATA SYNTHESIS: Immunogenetic, histopathologic, clinical, and electrophysiologic evidence was examined to explore the concept of HLA-B27-associated cardiac disease. RESULTS: Relative to the general population (in which the frequency is 6% to 8%), men (but not women) with pacemakers have a significantly increased frequency of HLA-B27. Furthermore, a cardiac syndrome that consists of severe conduction system abnormalities plus aortic regurgitation is associated with HLA-B27, which was present in 67% to 88% of the patients with both of these clinical findings. This proportion of HLA-B27 is similar to that found in patients with ankylosing spondylitis and Reiter disease. Cardiac and aortic tissue both show intimal proliferation of small arteries (obliterative endarteritis) and fibrosis, as seen in tissues adjacent to afflicted joints. Both cardiac conduction abnormalities and aortic regurgitation occur in patients with various HLA-B27-related extracardiac disorders, regardless of the severity of the latter; in about 50% of patients with these cardiac findings, a diagnosis of an HLA-B57-related rheumatic disease has not previously been made. Third-degree (complete) atrioventricular block related to HLA-B57 is located within the atrioventricular node in 95% rather than the expected 20% of cases. CONCLUSIONS: The heart and the joints are both of major importance as targets for the HLA-B57-associated disease process. HLA-B57-related cardiac lesions may be found in the absence of other rheumatologic manifestations. Furthermore, a genetically linked cardiac syndrome has been defined: the combination of conduction system abnormalities and aortic regurgitation.

Arrhythmias, Cardiac↗

Vascular damage caused by Cruorifilaria tuberocauda in the capybara (Hydrochoerus hydrochaeris).

Infection with Cruorifilaria tuberocauda caused vascular damage in the kidneys, lungs and heart of the capybara (Hydrochoerus hydrochaeris). The most significant lesions observed were in the renal and pulmonary arteries. They consisted of villous endarteritis, intimal and medial hypertrophy of the vessel walls, and large rugose protuberances that encroached upon the lumen leading to pyramidal infarcts in the kidneys. Coronary vascular lesions were related to dead and calcified parasites.

Animals↗

Spirorchidiasis and fibropapillomatosis in green turtles from the Hawaiian Islands.

Pathologic examination of green turtles (Chelonia mydas) from the Hawaiian Islands (USA) was performed to determine the primary cause of mortality. Lesions were associated with fibropapillomatosis (FP) and/or spirorchidiasis (SP) in 16 of 17 green turtles examined. Gross lesions included moderate to severe emaciation, lobulated fibropapillomas of different size classes, serous atrophy of fat, and edema of subcutaneous tissue and muscle. Anasarca, hydropericardium and pulmonary edema were common findings. The neoplastic lesions observed in the gastrointestinal tract, lungs, liver, and kidneys of 29% of turtles examined were histologically characterized as fibromas. A generalized thickening and hardening of major vessels and thrombosis with partial or complete lumen occlusion were observed in turtles with FP and SP. Histologically, lymphoplasmocytic endarteritis was observed in vessels of turtles with both conditions. Multifocal granulomas were associated with trematode ova in the parenchyma of most organs of all turtles with FP and SP. Spirorchidiasis and FP were considered the primary causes of mortality in the turtles examined. Further studies should focus on the pathogenic interaction of both conditions and their synergism as debilitating and fatal diseases in this threatened species.

Animals↗