1/6. Cholinsalicylate gel induced oral lesion: report of case.salicylic acid and its derivatives are extensively used medications for the treatment of systemic and local diseases. However, injudicious use of aspirin as well as other derivatives of salicylic acid, may cause systemic and oral complications such as mucosal burns and oral ulcers. In children, topical administration of these drugs, even in small dosages, may cause adverse reactions. This report shows a case of an 8 year old boy with G6PD deficiency, who had a mucosal burn caused by application of a cholinsalicylate paste. Three days later, the child developed oral ulcers, malaise and fever. The present case is characteristic of the enigmatic nature of the etiology and diagnosis of oral lesions, and the possible connection between cholinsalicylate systemic absorption and hemolytic anemia is discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/6. Chronic parvovirus infection and G6PD deficiency masquerading as diamond-Blackfan anemia.bone marrow aplasia due to parvovirus B19 infections is usually mild and self-limited. In patients with hereditary or acquired hemolytic anemias, B19 infection can cause a severe and life-threatening anemia due to the shortened half-life of red cells, but here, too, the transient nature of the infection soon remits the symptoms. Chronic infections with parvovirus are more characteristically associated with immunodeficiency states. We report here a case of B19-induced anemia in a patient with G6PD deficiency and hypogammaglobulinemia which was mislabeled as diamond-Blackfan anemia until the use of modern laboratory techniques allowed a correct diagnosis to be made.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/6. Vitreoretinal hemorrhages after ingestion of fava beans in a G-6-PD-deficient subject.A case of vitreo retinal hemorrhages following a hemolytic crisis by fava beans in a G-6-DP-deficient patient is reported. Intravascular coagulation due to thromboplastin-like substances liberated by the diseased RBC could be the cause. The possibility of vitreoretinal hemorrhages of this nature in young subjects from the areas where G-6-PD deficiency is endemic is stressed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/6. Haemolysis in a G6PD-deficient child induced by eating unripe peaches.A child suffering from G6PD deficiency developed a severe haemolytic crisis without an apparent trigger. The possible pathogenetic role of the ingestion of unripe peaches was studied biochemically in this anaemia. We show that an extract from the unripe peach exerts an oxidative challenge on normal as well as on asymptomatic G6PD-deficient erythrocytes. This effect is analogous to that of the favism-inducing agents. The effect of the extract on the patient's red blood cells was more pronounced than on other asymptomatic G6PD-deficient erythrocytes, particularly during his haemolytic crisis. The chemical nature of the deleterious component was not identified. It is suggested that unripe peaches be added to the list of hazards for G6PD-deficient subjects in combination with other factors.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/6. Protoporphyrin (proto)-determined hepatopathy in a South African Jewish family.A detailed study of a Jewish erythrohepatic protoporphyria (EHP)-affected family with a sibship consisting of 2 brothers and a sister, all of whom manifested protodetermined hepatopathy which varied from relatively mild hepatic involvement in the sister to a fulminant fatal illness in the eldest brother. The nature and course of his illness as well as the autopsy findings are described in detail. This sibship is also unique in that the 2 brothers and biochemical evidence of a severe degree of G6PD deficiency, while the sister was shown to be a carrier.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/6. wiskott-aldrich syndrome: cellular impairments and their implication for carrier detection.A family in which two male siblings were affected with wiskott-aldrich syndrome (WAS) was studied using G-6-PD isoenzymes as an X-linked marker in order to investigate the nature of cellular abnormalities. Isolated peripheral blood cell types from the doubly heterozygous mother of the affected males seemingly failed to express the G-6-PD allele in cis position with the WAS allele while her cultured skin fibroblasts expressed both G-6-PD alleles. Additionally, a histogram analysis of platelet size revealed a single population of abnormally small platelets in the affected propositus, whereas the heterozygous mother had no appreciable small platelet subpopulation. in vitro culture of hemopoietic progenitor cells of the heterozygous mother showed that the majority of progenitor cells did not express the WAS allele. However, a small number of cells expressing the G-6-PD type linked with the WAS allele were detected. The proportion of the latter progenitors was significantly higher among more primitive progenitors (those giving rise to later appearing colonies). This observation suggests that selection against cells expressing the Wiskott-Aldrich defect takes place in the hemopoietic system of the heterozygous female and offers a possible means of carrier detection in some women. Linkage studies in this family revealed one example of probable recombination between the loci for WAS and G-6-PD among three informative subjects, suggesting that these two loci may not be closely linked on the X-chromosome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |