Presse Med
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Hereditary iron overload is mainly due to mutations of the HFE gene, implicated in most cases of hereditary hemochromatosis. Non-HFE-related hereditary iron overload is rare. It includes hereditary hemochromatosis related to mutations of other genes, ferroportin disease (also known as hemochromatosis type 4), and entities associated with specific clinical manifestations. ⋯ Ferroportin disease is a special dominantly inherited clinical form of iron overload due to mutations of the SLC40A1 gene. Its expression differs significantly from that of hereditary hemochromatosis, and its mechanism is related to impairment of iron release from reticuloendothelial cells. Other causes of non-HFE-related hereditary iron overload are usually associated with recognizable clinical manifestations, such as anemia or neurological disorders.
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Comparative Study
[Helicobacter pylori infection and eradication are not related to glycosylated hemoglobin levels (HbA1c) in young patients with type 1 diabetes].
Helicobacter pylori (Hp) is a chronic stomach infection common throughout the world. The pediatric diabetes literature on the relation between Hp and HbA1c is sparse and controversial. This study aimed to investigate this relation. ⋯ Before treatment, patients seropositive for Hp did not differ in HbA1c levels or abdominal complaints according to whether they had active Hp infection. Hp infection was twice as frequent in Moghrabin Caucasians than in European Caucasians, perhaps associated with their lower socioeconomic status. HbA1c levels were similar in patients with and without Hp eradication after one treatment. After a one-year follow-up, Hp eradication had no significant effect on HbA1c levels.
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The two most frequent endocrine complications of hemochromatosis are diabetes mellitus and hypogonadotrophic hypogonadism. Other endocrine disorders related to this disease are very rare and are described especially in the most severe and earliest posttransfusion iron overloads. Endocrine complications are evidence of advanced hemochromatosis, often already associated with cirrhosis. ⋯ Testing for iron overload is recommended in subjects with atypical type 2 diabetes (for example, patients who are not overweight) and in cases of hypogonadism, characteristic pigmentation, or cirrhosis. Phlebotomy plays an important role in the management of endocrine complications of hemochromatosis, especially when diagnosis is early. In all cases of hypogonadotrophic hypogonadism, primary hemochromatosis must be considered.
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Nurses play a crucial role in end-of-life care, especially for outpatients. It is important to assess their knowledge and attitudes regarding pain management during the end-of-life period. ⋯ Attitudes toward pain management at the end of life do not depend only on book learning. They are also influenced by more subjective factors that may cause inequality in care for women and the elderly. Nurse training programs should take such factors into account.