Non-recordable electroretinogram in siderosis bulbi might not indicate poor visual outcome.
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A wide range of topics can be covered when considering a review of respiratory therapeutics. This review focuses on advances and controversies in the therapy of asthma, including issues regarding medications such as inhaled beta 2-agonists, inhaled corticosteroids, cromolyn sodium, and theophylline. Bronchodilator therapy for acute viral bronchiolitis remains a controversial issue and is discussed in light of recent published manuscripts. Issues regarding surfactant therapy for respiratory distress syndrome remain prominent in the neonatal respiratory therapeutics literature and recent findings in this area are reported. Advances in the treatment of cystic fibrosis, as well as a review concerning the pulmonary toxicity of various medications used in the treatment of pediatric illness are discussed.
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No matter how little the amount of blood and no matter how infrequent the event, the presence of hemoptysis is a terrifying and infernal experience for the patient. For the physician the evaluation of pulmonary bleeding carries an urgency that requires a logical plan and prompt execution.
The pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare, low-grade neoplasm that features atypical stromal cells containing hemosiderin, partially thrombosed ectatic vessels with circumferential hyalinization, and a variable inflammatory infiltrate. Over the years, we have occasionally observed a monomorphic partially myxoid spindle cell component (provisionally termed "early PHAT") co-existing with classic PHAT. In some instances, this monomorphic lesion occurs by itself, suggesting an early stage in the evolution of PHAT. To explore this hypothesis and to better define the long-term behavior of PHAT, we have reviewed our experience with these lesions. Forty-one cases were identified from consultation files. They occurred chiefly in adults (median 51 years, range 10-79 years) of either sex (23 female, 18 male), ranged from 0.3 to 19.7 cm (median 5.6 cm), and involved the subcutis of the ankle/foot (N = 15), leg (N = 10), thigh (N = 6), and other sites. Thirteen tumors had been present for more than 1 year prior to biopsy. Fifteen tumors consisted entirely of typical PHAT. Twelve cases conformed to "early PHAT," lacked fully developed features of PHAT, and showed instead short fascicles of hemosiderin-stippled spindled cells that infiltrated fat and surrounded congeries of small, damaged vessels. With close scrutiny, all contained rare pleomorphic cells with intranuclear pseudo-inclusions, as seen in typical PHAT. Fourteen cases contained both classic and early PHAT, in variable proportions. Follow-up was available in 18 patients (mean 84 months, median 52 months; range 13-420 months). Six of 18 patients developed recurrences, 1 with classic PHAT, 1 with mixed early-classic PHAT, and 4 with early PHAT. Following resection, 16 are disease free and 2 have persistent disease. None has developed metastasis. Based on the histologic overlap between early and classic PHAT, the presence of early PHAT at the periphery of classic PHAT, and the admixture of both patterns within the same tumor, we conclude that early PHAT represents a precursor lesion for PHAT. It appears essentially identical to the lesion termed "hemosiderotic fibrohistiocytic lipomatous lesion," supporting the notion that the latter is a neoplastic rather than a reactive lesion. PHAT should be considered mesenchymal tumors of intermediate malignancy, given their high rate of (sometimes aggressive) local recurrence.
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The peri-operative anaesthetic management and hazards of a patient with idiopathic pulmonary haemosiderosis are described in association with the relevant known clinical and pathological data.
An ultrastructural study of eccrine sweat glands was carried out on three patients with iron overload (two with idiopathic haemochromatosis and one with transfusional haemosiderosis). In all three patients small electron-dense particles were seen in both eccrine sweat glands and macrophages. They appeared primarily in the clear cells of the eccrine sweat glands as membrane-bound irregularly shaped bodies, and were shown to contain iron by energy dispersive X-ray microanalysis. Iron aggregates in and around sweat glands were also seen on light microscopy.
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We have encapsulated desferrioxamine (DF) in multilamellar liposomes (ML) and unilamellar liposomes (UL). Liposomes were prepared either with or without a glycolipid, i..e. galactocerebroside (GC). The average diameter of ML was 0.5 microgram, and that of UL was 0.08 microgram. Less tha 5% of DF leaked out from the liposomes after incubation in mouse plasma for 6 h. 59Fe-ferritin and 59Fe-labelled heat damaged erythrocytes (59Fe-DRBC) were administered to normal and hypertransfused mice as models of iron overload. Ferritin was used to label liver parenchymal cells and DRBC to label the Kupffer cells of the liver. A single injection of ML or UL with or without galactocerebroside into normal and hypertransfused mice enhanced from 3- to 15-fold the urinary excretion of radioiron from 59Fe-ferritin and from 59Fe-DRBC injected mice. For both the normal and hypertransfused mice, liposomes containing GC removed more 59Fe radioactivity from mice receiving 59Fe-DRBC. Thus GAC-liposomes may have a higher affinity for parenchymal cells of the liver, whereas liposomes without the glycolipid may have a higher uptake by the Kupffer cells.
This report updates the combined experience of four centres involved in the long-term treatment of transfusional iron overload in 84 patients with the oral iron chelator deferiprone (L1) over 167 patient-years. The source of L1 was variable, including two university research laboratories and three pharmaceutical firms. Compliance was rated as excellent in 48%, intermediate in 36%, and poor in 16% of patients. On a mean L1 dose of 73-81 mg/kg/d, urinary iron excretion was stable, at around 0.5 mg/kg/d, with no indication of a diminishing response with time. Serum ferritin showed a very steady decrease with time from an initial mean +/- 1 SD of 4207 +/- 3118 to 1779 +/- 1154 micrograms/l after 48 months (P < 0.001). 17 patients abandoned L1 therapy. Major complications of L1 requiring permanent discontinuation of treatment included agranulocytosis (three), severe nausea (four), arthritis (two) and persistent liver dysfunction (one). The remaining patients abandoned treatment because of low compliance (three) and conditions unrelated to L1 toxicity (four). Lesser complications permitting continued L1 treatment included transient mild neutropenia (four), zinc deficiency (12), transient increase in liver enzymes (37), moderate nausea (three) and arthropathy (17). There was no treatment-related mortality. Although the complications associated with L1 treatment are significant and require close monitoring, they do not preclude effective long-term therapy in the vast majority of patients. Further well-controlled prospective studies of L1 are required in order to enable proper judgement of its suitability for general long-term clinical use.
Total body iron stores including liver and spleen iron were assessed by non-invasive SQUID biomagnetometry. The liver iron concentration was measured in groups of patients with beta-thalassaemia major or other posttransfusional siderosis under treatment with the oral iron chelator deferiprone (n = 19) and/or with parenteral deferoxamine (n = 33). An interquartile range for liver iron concentrations of 1680-4470 micrograms/g liver was found in these patients. In both groups a poor correlation between liver iron and serum ferritin values was observed. Repeated measurements of liver and spleen iron concentrations as well as determination of liver and spleen volume by sonography were performed in six patients under continuous deferiprone treatment for 3-15 months. In this group detailed information was obtained on the whole body iron store (5-36g) and the iron excretion rates (14-34 mg/d) for each patient. As indicated by decreasing liver iron concentrations, five out of six subjects showed a negative iron balance (2-13 mg/d). Conventional measurements of both serum ferritin and urine iron excretion gave fluctuating results, thus being only of limited use in the control of iron depletion therapy. The non-invasive biomagnetic liver iron quantification is a precise and clinically verified technique which offers more direct information on the long-term efficacy of an iron depletion therapy than the hitherto used methods. This technique may be of use in the clinical evaluation of new oral iron chelators.
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Targetoid haemosiderotic haemangioma represents a new, rarely reported, distinctive, benign vascular tumour, characterized histopathologically by a biphasic growth pattern of dilated vascular structures in the superficial dermis lined by prominent hobnail endothelial cells and collagen dissecting, rather narrow neoplastic vessels in deeper parts of the lesion. In the initial stage, the lesion is seen as a small purple or violaceous papule, 2--3 mm in diameter. Over time, the ecchymotic ring expands peripherally until it disappears spontaneously. In the later stages, however, the central papule remains as a slightly raised dermal lesion with a purple to brownish discolouration. We report three cases whose repetitive cyclic morphological changes of targetoid haemosiderotic haemangiomas were monitored dermoscopically at 3-month follow-ups. Histopathological examination of each lesion identified the features of targetoid haemosiderotic haemangioma. To the best of our knowledge, our three cases are the first reported in the literature of targetoid haemosiderotic haemangiomas that were regularly monitored by dermoscopic examinations, enabling development of the different stages of the same lesion to be followed.