Hemolytic anemia in Wilson's disease.
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Rapidly progressive bilateral pulmonary infiltrates occurred in a 19-year-old man following an unusual hydrocarbon abuse. The acute illness was the result of a "trick" known as "fire-breathing." Fire-breathing involves filling the oral cavity with butane gas, from an ordinary butane cigarette/cigar lighter, and exhalation of the volatile vapors over an open flame producing a flame-throwing effect. Because of the pulmonary toxic reaction, this activity could have a serious or even fatal outcome.
The fatty acid composition of membrane phospholipids of stimulated and unstimulated platelets was studied in six normal volunteers given a daily dietary supplement of a fish oil rich in eicosapentaenoic acid (EPA) for 4 weeks. The supplement was equivalent to 1.8 g of EPA daily. Thromboxane synthesis and platelet aggregation responses to sodium arachidonate, thrombin and the ionophore A23187 were also investigated. A marked increase in the relative EPA content of phosphatidylcholine (PC) and phosphatidylethanolamine (PE) was noted after 2 and 4 weeks fish oil supplementation. However, there was no incorporation into phosphatidylinositol (PI) or phosphatidylserine (PS). The relative arachidonic acid (AA) content of PC and PE was significantly reduced at 2 and 4 weeks but that of PI and PS remained unchanged. Significant reductions in the relative linoleic acid content of total phospholipids, PC and PE were also noted. Stimulation of platelets obtained after 4 weeks fish oil supplementation by thrombin and A23187 was associated with a marked reduction in the AA content of PI and a minor reduction in that of PE. There was no change in the relative proportions of EPA in PI, PS, PC or PE after stimulation. Throughout the study there were no significant changes in platelet aggregation responses or in platelet thromboxane production. Our results indicate that the incorporation of EPA into the platelet membrane phospholipids is selective and that if PI is the major source of AA for platelet prostaglandin biosynthesis then the reported beneficial effects of EPA on haemostasis cannot be explained on the basis of its incorporation into and mobilization from the platelet membrane phospholipid pool.
PURPOSE: To report the use of computed tomographic (CT) guidance for percutaneous treatment of an isolated internal iliac artery (IIA) aneurysm after open aortic aneurysm repair. CASE REPORT: A 74-year-old man presented with an isolated IIA aneurysm 8 years after an open repair of his abdominal aortic aneurysm. In view of his diabetes, hypertension, and chronic renal impairment, an endovascular technique was selected. However, because of previous ligation of the internal iliac origin, a transarterial approach could not be used. The proximity of the aneurysm to the anterior abdominal wall allowed us to gain access to it percutaneously using CT guidance to perform embolization. CONCLUSION: CT-guided direct puncture of isolated IIA aneurysms adds to the current armamentarium of minimally invasive modalities. It is a technique that can be applied to isolated IIA aneurysms that develop subsequent to AAA repair or appear in cases where intra-arterial access is not possible.
A surgical technique of partial detrusorectomy (autoaugmentation) for creation of a diverticular bulge of the dog bladder is described. The method is designed to serve as a bladder augmentation without using bowel. We describe the technique, results with dogs and its clinical application. Partial detrusorectomy appears to function as an autoaugmentation that can be performed reliably in dogs, offers potential advantages over bowel segments, and is a potential alternative to enterocystoplasty in selected patients.
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Aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum, severe mental retardation, and a characteristic chorioretinopathy with lacunar defects. The authors report on a 2-year-old girl with congenital hydrocephaly who was found unresponsive by the baby-sitter and died shortly thereafter. At autopsy, the histopathologic findings, which were confined to the brain and eyes, were found highly characteristic of AIC. The main abnormalities included agenesis of the corpus callosum, micropolygyria, bilateral papillomas of choroid plexi, bilateral microphthalmia, bilateral hypoplasia of the optic nerves, bilateral colobomas of the juxtapapillaris choroid and optic disc, bilateral total retinal detachment with dysplastic rosettes and chorioretinal lacunae with focal thinning, and atrophy of the retinal pigment epithelium and choroid. A detailed histopathologic study of the ocular findings and the brain anomalies is presented. The results of scanning electron microscopy of the chorioretinal lacunae demonstrated peculiar papillary proliferations of the retinal pigment epithelium in both eyes.
We have investigated platelet morphology, function and biochemistry in a family affected by dominantly inherited congenital thrombocytopenia with giant platelets. A defect of aggregation was defined with evidence of disturbed fatty acid distribution in platelet membrane phospholipids and impaired arachidonic acid mobilization. Release of 5-hydroxytryptamine was normal. Platelet ultrastructure was grossly abnormal and volume analysis revealed small as well as giant forms. Bone marrow megakaryocytes were morphologically abnormal and the distribution of mean nuclear ploidy atypical, suggesting the production of abnormal platelets due to a primary megakaryocyte disorder. The features of this condition are distinct from those previously described in familial thrombocytopenia and constitute a new "giant platelet syndrome". The qualitative and quantitative platelet defects may be secondary to a disturbance of megakaryocyte cytoplasmic fragmentation.
Benign hepatic tumors and peliosis hepatis developed in a patient who had received androgen therapy for three years. The tumors were identified by arteriography. Peliosis hepatis was diagnosed by wedged hepatic venography: to our knowledge, this method has not been previously used to diagnose this condition. Wedged venography, performed four months after withdrawal of androgen therapy and after liver function had nearly returned to normal, demonstrated morphologic improvement as well. On the basis of this case and other histologic descriptions of peliosis hepatis, we believe that wedged hepatic venography is a simple, sensitive, and specific diagnostic modality.
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A family with dominantly inherited short stature, short broad hands and feet, normal facial appearance and normal intelligence is described. Radiographs demonstrated features of brachydactyly type E. A differential diagnosis of brachydactyly is given.
Meningococci continue to cause outbreaks of disease throughout the world. The UK is currently experiencing an upsurge due to organisms of groups B and C. There is substantial under-reporting of meningococcal disease, only meningitis currently being notifiable. All significant meningococcal strains should be typed by a reference laboratory. New approaches to vaccine development permit cautious optimism for the future.
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Two neonates with the Kasabach-Merritt syndrome (cavernous haemangioma and spontaneous bleeding) are described. The first bled into a rapidly growing cervical haemangioma. A consumptive coagulopathy was found, and he required blood transfusions; no response to steroids was observed. Further treatment was conservative, and at the age of 2 years he is well and the haemangioma is shrinking. The second infant had a strawberry naevus on the thigh which started bleeding when he was 10 days old, a buttock mass and a mass in the foot, thought to be lipomas. No clear benefit was derived from steroids. Bandaging of the thigh prevented further bleeding, and he is well at the age of 10 months. Conservative management seems best in the condition, and steroids appear to be of value in approximately 40% of cases.
The use of intrathecal diamorphine via an implanted portal system is described for pain control in a patient suffering from vertebral metastatic disease. The complication of myoclonic spasms affecting the lower half of the body occurred after 14 days, when increasing the bolus dose to 40 mg. The spasms lasted for 3 hr and then gradually subsided. Diamorphine was subsequently restarted at a lower dose of 15 mg twice daily. On increasing the dose to 20 mg diamorphine 10 days later, severe distressing myoclonic spasms recurred 20 min postinjection. Myoclonus could only be controlled by instituting a local anesthetic intrathecal block. The patient was finally managed with 20 mg diamorphine per day by intrathecal infusion, and the pain was reasonably well controlled for the following 10 weeks without any recurrence of myoclonic spasms.
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It is frequently difficult to identify and localize organic intraorbital foreign bodies despite modern day high-resolution imaging studies. Although there can be grave complications associated with retention of organic intraorbital foreign bodies, many believe that removal of such bodies in most cases is unwarranted. A high clinical suspicion, proper choice of imaging studies, and removal by a skilled orbital surgeon probably make the risk of surgical exploration and foreign body removal less than the risk of foreign body retention. We present a case of an intraorbital wood foreign body that required two separate explorations for retrieval. An initial intraconal exploration failed to locate the foreign body. Although the clinical suspicion was high, the imaging studies were equivocal, complicating the management. A second exploration yielded a large intraorbital wooden foreign body in the inferior extraconal space. The patient fully recovered and regained visual acuity of 20/20. The evaluation of such patients and details of management strategy are discussed.
A woman with a history of bilateral mastectomy and silicone implants for fibrocystic disease and a history of atrial septal defect repair presented with pleural nodules on a chest radiograph. A thorascopic biopsy performed for possible mesothelioma demonstrated chronic inflammation and focal pleural fibrosis due to a foreign-body reaction secondary to silicone. This was confirmed using scanning electron microscopy and energy-dispersive radiograph elemental analysis. As the population ages, the increasing frequency of ruptured silicone implants and the need for heart surgery may result in a corresponding increase in the risk for fibrothorax secondary to inadvertent silicone introduction during surgery.