Cine-MRI-aided endomyocardectomy in idiopathic hypereosinophilic syndrome

The idiopathic hypereosinophilic syndrome is a leukoproliferative disorder marked by a predilection to damage specific organs, including the heart. This report describes a patient with extensive endocardial fibrosis accompanying this syndrome. Right ventricular endomyocardectomy with preservation of the tricuspid valve was performed. The procedure was aided by cine-magnetic resonance imaging for preoperative assessment and follow-up of surgical results.     

The interleukin-5 messenger RNA expression in a patient with idiopathic hypereosinophilic syndrome

Interleukin-5 has a specific role in various eosinophilic activities. It is the predominant cytokine produces by activated T-lymphocytes isolated from patients with idiopathic hypereosinophilic syndrome. We studied a young patient suffering from idiopathic hypereosinophilic syndrome who presented with Horner's syndrome, peripheral neuropathy and skin ulcers. The IL-5 gene expression by CD4+ T-lymphocytes and the peripheral eosinophil count were raised. The skin ulcers continued to deteriorate despite a swift reduction of the IL-5 gene expression and peripheral eosinophil count following systemic corticosteroid treatment. We suggest that peripheral eosinophilia may not be responsible for the damage in skin lesions and more aggressive treatment may be required.     

Interferon-alpha in the idiopathic hypereosinophilic syndrome: consideration of five cases

A consortially developed continuing education series for rural nurses was implemented using videoconferencing technology. This article provides an overview of distance education technologies, the history and goals of the demonstration project at Edinboro University, and the coordination activities and instructional issues faced by the faculty. Participants became acquainted with distance education technology as they developed a process for program development, delivery, evaluation, and for awarding continuing education credits. The project was well received by instructors and students and testified to previous findings in the literature that distance education is a viable delivery system in terms of a teaching tool, learner satisfaction, and cost effectiveness.     

Idiopathic hypereosinophilic syndrome with eosinophilic myositis, peripheral neuropathy and central nervous system involvement

Idiopathic hypereosinophilic syndrome (HES) is a rare disorder marked by a sustained overproduction of eosinophils and a predilection for damage to multiple organ systems. Its neurologic involvement ranges from the central to the peripheral nervous system, and can be associated with eosinophilic myositis. We report a 68-year-old woman who had eosinophilia, eosinophilic dermatitis and eosinophilic pneumonia. She also suffered from numbness and weakness of the lower limbs. Because of long-lasting (> 6 mo) eosinophilia (> 1.5 x 10(9)/L) in the peripheral blood and the fact that no other underlying causes of eosinophilia and neurologic involvement could be identified, a diagnosis of idiopathic hypereosinophilic syndrome was made. The muscle biopsy showed infiltration of inflammatory cells, including a few eosinophils (Liu's stain). Magnetic resonance images, motor evoked potentials, somatosensory evoked potentials and nerve conduction velocities also showed abnormalities in the central and peripheral nervous systems. The pathogenesis and treatments of HES are discussed in this report.     

Sarcoidosis with cardiac involvement

Patients with significant cardiac sarcoidosis are at increased risk of sudden death from ventricular dysrhythmias or conduction disturbances. We report a patient in whom there was radiographic and histologic evidence of systemic sarcoidosis; though histologic confirmation of involvement of heart by sarcoidosis is lacking, the clinical manifestations, radionuclide image findings, rhythm disturbances, and the response to steroid therapy are strong evidence in favor of myocardial involvement by the granulomatous process.     

A unique pulmonary venous abnormality: with associated pulmonary arterial anomaly and arteriovenous malformation

Lead and cadmium were administered intraperitoneally, singly and jointly, to the mice. The levels of cadmium, copper, manganese, lead and zinc were determined in liver, kidney and brain by atomic absorption spectrophotometric technique and delta-aminolevulinic acid dehydratase (ALA-D) activity was determined in erythrocytes. The tissue levels of some of these metals were found significantly altered by cadmium and lead both, but cadmium was found to have no effect on blood on ALA-D activity.     

A case of hypereosinophilic syndrome associated with factor V Leiden mutation and thrombosis

The authors present a case of serotonin syndrome in a prisoner who was transferred to a psychiatric hospital because of increasing psychotic symptoms. They discuss some factors that appear to put some populations at higher risk for such syndromes, and recommend increased vigilance for such problems in those identified populations.     

Necrobiotic xanthogranuloma with cardiac involvement

A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.     

Allogeneic bone marrow transplantation for hypereosinophilic syndrome with advanced myelofibrosis

A 33-year-old man with an atypical course of hypereosinophilic syndrome including malignant hypercalcemia, osteolytic lesions and evolution into severe myelofibrosis was treated by allogeneic bone marrow transplantation after conditioning with cytoxan and total body irradiation. As the transplant was sex-mismatched, chimerism was studied by means of cytogenetic analysis and Y chromosomal DNA amplification by PCR assay. Long-term complete remission has been assessed by normalization of blood cell counts, magnetic resonance imaging and karyotypic analysis. A relapse was observed 40 months after transplantation. The patient remains alive 44 months post-BMT. This case report is compared with those reported in the literature.     

Life-threatening cardiac involvement throughout life in a case of Costello syndrome

A virus isolated from vaginal exudate of cows with vaginitis showed molecular weight around 92 to 143 x 10(3) KD, density between 1.168 and 1.203 g/ml and physical, chemical and morphological properties characteristic of virus belonging to the herpes group. This virus was not reagent with the fluorescent antibodies of Infectious Bovine Rhinotracheitis (IBR) or Parainfluenza-3 (PI-3) viruses, frequently detected in cows with vaginitis.     

Cardiomyopathic lentiginosis/LEOPARD syndrome presenting as sudden cardiac arrest

A 26-year-old apparently healthy man with numerous pigmented skin lesions collapsed during an evening party and was resuscitated from ventricular fibrillation. Hypertrophic cardiomyopathy and subaortic tunnel were disclosed by angiocardiography. A diagnosis of cardiomyopathic lentiginosis/lentigines (multiple), electrocardiographic abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth, and deafness (sensorineural) syndrome was made. The patient then underwent treatment with an implantable pacer-cardioverter-defibrillator device. Further evaluation revealed several well-established features of the disorder. This is the first reported case of survival from ventricular fibrillation associated with this rare and little known multifaceted syndrome. Disseminated lentiginosis must prompt clinicians to evaluate such cases further since underlying disorders may be associated with considerable morbidity and, apparently, sudden death.     

Nesidioblastosis-associated hypoglycaemia presenting with prominent cardiac manifestations

We present a case of nesidioblastosis presenting with hyperinsulinaemic hypoglycaemia and electrocardiographic abnormalities.