Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH). Glucocorticosteroids (CS) represent the first line therapy for IPH. Although most patients respond to CS, steroid refractoriness is seen in an appreciable minority of patients. This paper reviews and evaluates the efficacy and safety profile of liposomal dexamethasone 21-palmitate (liposteroid) for the treatment of IPH. Medline, Embase and Web of Science biomedical databases were searched between 1980 and 2020 to identify papers describing patients with IPH, who were treated with liposteroid. A total of five articles were identified. Four in the form of case reports and one as a case series. A total of 12 pediatric patients (5 boys, 7 girls) were identified, with a median age of 2.3 years (range 0.5-8.6). Liposteroid therapy in intravenous doses ranging 0.06-0.1 mg/kg body weight appeared to be effective for both remission induction therapy, and maintenance therapy. There was no mortality among patients treated with liposteroid, either in the acute phase or during follow-up. The majority of patients for whom long-term follow-up data were available, were cured or in disease remission. No acute adverse events were reported, and long-term side effects were minimal and tolerable. Liposteroid represents a potential alternative or supplement to conventional CS therapy, as it appears to be more efficacious and associated with fewer side effects. Larger prospective, controlled trials are necessary to be able to define more precisely the therapeutic role of liposteroid in IPH.

• Klíčová slova
• Dexamethasone, Dexamethasone 21-palmitate, Diffuse alveolar haemorrhage, Idiopathic pulmonary hemosiderosis, Therapeutic use,
• MeSH
• dítě MeSH
• hemosideróza * komplikace   diagnóza   farmakoterapie   MeSH
• kojenec MeSH
• lidé MeSH
• plicní hemosideróza MeSH
• plicní nemoci * komplikace   farmakoterapie   MeSH
• předškolní dítě MeSH
• prospektivní studie MeSH
• Check Tag
• dítě MeSH
• kojenec MeSH
• lidé MeSH
• mužské pohlaví MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• scoping review MeSH

This paper briefly reviews the safety and efficacy of liposteroid in different inflammatory and non-inflammatory diseases. Corticosteroids (CS) are the first-line therapy in many inflammatory and autoimmune disorders. Although highly efficacious, long-term use of CS is limited due to the occurrence of significant side effects. Liposteroid, which is a liposomal formulation of dexamethasone palmitate, possess more potent anti-inflammatory and immunosuppressive properties compared to dexamethasone sodium phosphate. These two formulations have markedly different lipid solubility, resulting in different pharmacokinetic and pharmacodynamic properties. Liposteroid has been used with success in patients with rheumatoid arthritis, macrophage activation syndrome, and idiopathic pulmonary hemosiderosis. In addition, liposteroid has been used in some non-inflammatory diseases. Moreover, we conceive that liposteroid may have a beneficial effect in patients, who are critically ill due to COVID-19, and suffer from the macrophage activation syndrome.

• Klíčová slova
• Autoimmune disease, COVID-19, Dexamethasone, Idiopathic pulmonary hemosiderosis, Liposome, Liposteroid,
• MeSH
• COVID-19 * MeSH
• glukokortikoidy MeSH
• hemosideróza * MeSH
• lidé MeSH
• plicní nemoci * MeSH
• SARS-CoV-2 MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH
• Názvy látek
• glukokortikoidy MeSH

Iron is an essential element for fundamental cell functions and a catalyst for chemical reactions. Three samples extracted from the human spleen were investigated by scanning (SEM) and transmission electron microscopy (TEM), Mössbauer spectrometry (MS), and SQUID magnetometry. The sample with diagnosis of hemosiderosis (H) differs from that referring to hereditary spherocytosis and the reference sample. SEM reveals iron-rich micrometer-sized aggregate of various structures-tiny fibrils in hereditary spherocytosis sample and no fibrils in hemochromatosis. Hematite and magnetite particles from 2 to 6 μm in TEM with diffraction in all samples were shown. The SQUID magnetometry shows different amount of diamagnetic, paramagnetic and ferrimagnetic structures in the tissues. The MS results indicate contribution of ferromagnetically split sextets for all investigated samples. Their occurrence indicates that at least part of the sample is magnetically ordered below the critical temperature. The iron accumulation process is different in hereditary spherocytosis and hemosiderosis. This fact may be the reason of different iron crystallization.

• Klíčová slova
• Diffraction, Iron, Magnetic properties, Spleen,
• MeSH
• dědičná sférocytóza metabolismus   patologie   MeSH
• hemosideróza metabolismus   patologie   MeSH
• krystalizace MeSH
• lidé MeSH
• oxid železnato-železitý chemie   MeSH
• pitva MeSH
• slezina chemie   metabolismus   patologie   ultrastruktura   MeSH
• spektroskopie Mossbauerova MeSH
• transmisní elektronová mikroskopie MeSH
• železité sloučeniny chemie   metabolismus   MeSH
• železo chemie   metabolismus   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• práce podpořená grantem MeSH
• Názvy látek
• ferric oxide MeSH Prohlížeč
• oxid železnato-železitý MeSH
• železité sloučeniny MeSH
• železo MeSH

Hemosiderotic fibrohistiocytic lipomatous lesion (HFLL) is a recently proposed lipomatous entity. HFLL was originally suggested to be a benign reactive lesion arising due to an antecedent trauma. We report two patients with HFLL who also suffered from chronic vein insufficiency due to varicose involving deep veins of the low limbs. Both patients were middle-aged women with solitary, poorly circumscribed subcutaneous lesions on the lower extremities. Histopathological examination revealed typical features of HFLL. We think that the consistent clinical features such as advanced age, female sex predilection, and specific location along with distinctive histopathological features allow the suggestion that impaired blood circulation, to wit, venous stasis is involved in the pathogenesis of HFLL. We hypothesize that the proliferation of spindled fibroblastic and myofibroblastic cells and capillaries, erythrocyte extravasation, and hemosiderin deposition with lipomatous tissue of HFLL may simply represent an exaggerated tissue response to venous stasis in which elevated venous and capillary pressures, oxygen saturation, and edema stimulate the proliferation of the above mentioned elements and lead to erythrocyte extravasation. A similar histopathological pattern is seen in acroangiodermatitis of Mali and vascular transformation of lymph node sinuses, and these conditions are also associated with impaired blood circulation.

• MeSH
• hemosiderin analýza   MeSH
• hemosideróza komplikace   patologie   chirurgie   MeSH
• histiocytóza komplikace   patologie   chirurgie   MeSH
• imunohistochemie MeSH
• lidé středního věku MeSH
• lidé MeSH
• lipom komplikace   patologie   chirurgie   MeSH
• nádorové biomarkery analýza   MeSH
• nádory měkkých tkání komplikace   patologie   chirurgie   MeSH
• tuková tkáň chemie   patologie   MeSH
• varixy komplikace   patologie   chirurgie   MeSH
• výsledek terapie MeSH
• žilní insuficience komplikace   patologie   chirurgie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• hemosiderin MeSH
• nádorové biomarkery MeSH

Tissue damage, cardiac and hepatic failure are the most frequent consequences of chronic iron accumulation within the body. A long term administration of chelating agents may prevent organ damage by surplus of iron as well as improve cardiac and liver function in iron overloaded patients. Desferrioxamine (Desferal) is the only one chelating agent for routine clinical use. To produce a therapeutic effect parenteral administration of the drug in prolonged infusions is needed and therefore many investigators try to search for orally active chelator with effect comparable to desferrioxamine. Hydroxypyridones, especially 1,2-dimethyl-3-hydroxypyrid-4-one (L 1-Deferiprone), are the most intensively studied oral iron chelators. In animal and clinical studies L 1 administration caused iron excretion comparable to that obtained by desferrioxamine, however, some serious adverse effects (including agranulocytosis) related to L 1 treatment were observed. This problem still precludes wide large-scale clinical application of L 1. Other compounds possessing chelating activity after oral administration, eg. 1,2-diethyl-3-hydroxypyridone, PIH or HBED are also currently under investigation. Development of a safe inexpensive and orally effective iron chelator is the main objective of ongoing animal and clinical studies.

• MeSH
• chelátory železa terapeutické užití   MeSH
• deferoxamin terapeutické užití   MeSH
• hemosideróza farmakoterapie   MeSH
• lidé MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• práce podpořená grantem MeSH
• přehledy MeSH
• Názvy látek
• chelátory železa MeSH
• deferoxamin MeSH

Nine iron overloaded patients were treated with L1--Deferiprone (1,2-dimethyl-3-hydroxypyrid-4-one) in daily dose 3 g (40-50 mg/kg) for 12 weeks. In 7 patients the efficiency of L 1 treatment was compared to the therapeutic effect of the same dose of desferrioxamine (Desferal). A significant increase in urinary iron excretion was observed after administration of both chelating agents. Iron excretion after L 1 treatment was approximately 65% of that obtained with Desferal. The amount of excreted iron correlated with the amount of iron stores before chelation. A significant decrease in transferrin saturation, serum and red cell ferritin was observed after treatment with Desferal, L 1 administration caused a significant decrease only in serum ferritin level. However, all the parameters reflecting iron stores remained increased when compared to normal values after 12 weeks of chelation therapy. An incomplete absorption from gut and some reutilization of chelated iron may be responsible for less potent iron chelation by L 1 in comparison to Desferal. A low tolerance of the drug together with repeated nausea and vomiting were the most frequent adverse effects observed in the course of L1 administration. L 1 treatment had to be discontinued due to repeated vomiting in one patient and due to progressive granulocytopenia and thrombocytopenia in another patient. Because of the side effects more clinical studies with L 1 are needed before its introduction in wide clinical practice.

• MeSH
• aplikace orální MeSH
• chelátory železa aplikace a dávkování   MeSH
• deferipron MeSH
• deferoxamin terapeutické užití   MeSH
• hemosideróza farmakoterapie   MeSH
• lidé MeSH
• pyridony aplikace a dávkování   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH
• Názvy látek
• chelátory železa MeSH
• deferipron MeSH
• deferoxamin MeSH
• pyridony MeSH

Animals in groups of six dogs and six bitches were given daily in their food, individually, for a period of six months, the fungicide trimorphamide, at dosages of 0, 300 and 500 mg.kg-1 of their weight. After they had been killed, a significant reduction in their weight was observed along with hemosiderosis of the liver, kidneys and spleen of all the experimental animals. No other important structural changes were found by dissection and pathologico-histological examinations of almost all organs and tissues.

• MeSH
• hemosideróza chemicky indukované   patologie   veterinární   MeSH
• játra patologie   MeSH
• ledviny patologie   MeSH
• morfoliny otrava   MeSH
• nemoci psů chemicky indukované   patologie   MeSH
• psi MeSH
• slezina patologie   MeSH
• zvířata MeSH
• Check Tag
• psi MeSH
• zvířata MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• Názvy látek
• morfoliny MeSH
• trimorfamid MeSH Prohlížeč

• MeSH
• hemosiderin metabolismus   MeSH
• hemosideróza etiologie   metabolismus   patologie   MeSH
• kardiomyopatie etiologie   metabolismus   patologie   MeSH
• kongenitální hemolytická anemie terapie   MeSH
• lidé MeSH
• mladiství MeSH
• myokard metabolismus   patologie   MeSH
• potransfuzní reakce * MeSH
• Check Tag
• lidé MeSH
• mladiství MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• hemosiderin MeSH

• MeSH
• antibakteriální látky terapeutické užití   MeSH
• dítě MeSH
• hemosideróza diagnóza   farmakoterapie   MeSH
• lidé MeSH
• plicní nemoci diagnóza   farmakoterapie   MeSH
• prednison terapeutické užití   MeSH
• předškolní dítě MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• předškolní dítě MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH
• Názvy látek
• antibakteriální látky MeSH
• prednison MeSH

• MeSH
• aktivace lymfocytů * MeSH
• buněčná imunita * MeSH
• dospělí MeSH
• hemosideróza imunologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• sarkoidóza imunologie   MeSH
• silikóza imunologie   MeSH
• tuberkulinový test * MeSH
• tvorba protilátek MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH