Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
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Pol. Merkur. Lekarski · Apr 2018
[Mycophenolate mofetil (MMF) as the first choice immunosuppressive drug in treatment of steroid-dependent nephrotic syndrome in children].
Most children diagnosed with nephrotic syndrome show favourable response to corticosteroid therapy, nonetheless 30% of patients have frequent relapses or a steroid-dependent course of disease. Cyclophosphamide, cyclosporin A or MMF are being used in treatment of steroid-dependent nephrotic syndrome in search of a drug with highest long-term effectiveness and least amount of side effects.
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A diagnosis of pulmonary sarcoidosis is based on the assessment of clinical outcome, radiology findings and detection of noncaseating granulomas in cytology or histology specimens. Cytological material obtained from enlarged lymph nodes and/or histological specimens from bronchial mucosa and lung tissue are examined according to sarcoidosis stage. The most available are standard bronchoscopic methods as conventional transbronchial needle aspiration (cTBNA), endobronchial biopsy (EBB) and transbronchial lung biopsy (TBLB) both performed with use of forceps. ⋯ Considering a dynamic improvement in cytology assessment techniques (processed as cytology smears and cell blocks) the endoscopic methods with use of fine needle aspiration biopsy of enlarged lymph nodes became a method of choice in sarcoidosis with lymphadenopathy, and published data suggest a higher diagnostic yield when performed under endosonographic guidance. The optimal approach (transbronchial or transesophageal) and the selection of mediastinal lymph node stations considered for biopsy still need evaluation. Also TBLC, successfully used in the diagnosis of other diffuse parenchymal lung diseases, requires more experiences and trials to establish its role in diagnosis of pulmonary sarcoidosis.
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Pol. Merkur. Lekarski · Mar 2018
Review[Neurosarcoidosis - diagnosis, clinical picture and therapy].
Sarcoidosis (SA) is a granulomatous, multisystem disease of unknown etiology. Most often the disease affects lungs and mediastinal lymph nodes, but it may occur in other organs. Neurosarcoidosis (NS) more commonly occurs with other sarcoidosis forms, in 1% of cases it involves only nervous system. ⋯ Corticosteroids still are the first line of treatment for NS patients. In cases of steroids resistance, lack of their effectiveness or existence of contraindication to their use, immunosuppressant treatment is recommended. The latest NS algorithm with immunosuppressive treatment is discussed.
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Pol. Merkur. Lekarski · Mar 2018
Review[Nintedanib in the treatment of fibrosing interstital lung diseases].
Nintedanib is an intracellular tyrosine kinase inhibitor approved in a treatment of idiopathic pulmonary fibrosis. It reduces the annual rate of forced vital capacity decline by approximately 50%, that results in slowing of disease progression. The drug also reduces the incidence of acute exacerbations of idiopathic pulmonary fibrosis. ⋯ The research on combination therapy with pirfenidone did not show the new adverse drug reactions, there were no changes in pharmacokinetics of both drugs. A trend to improve the efficacy of the combination therapy in comparison to monotherapy with both drugs was noted, but further research in this field is needed. There are also trials of nintedanib in the treatment of other fibrosing interstitial lung diseases, i.e. chronic hypersensitivity pneumonitis, stage IV sarcoidosis or connective tissue disease related lung disorders.
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Pol. Merkur. Lekarski · Mar 2018
Review[Disturbances of calcium metabolism and vitamin D supplementation in sarcoidosis - two-way street].
The role of vitamin D in the human body is not limited only to the regulation of calcium metabolism and secondary to the impact on bones. Recent studies have shown the influence of vitamin D level on muscles, on the risk of cancer, diabetes, hypertension and pulmonary diseases, including granulomatous diseases. Sarcoidosis is a granulomatous disease of unknown etiology. ⋯ Vitamin D supplementation, according to current recommendations for general population, is based solely on 25-hydroxyvitamin D level testing. This seems to be not safe in the group of sarcoidosis patients. This article discusses the role of vitamin D in sarcoidosis patients and current opinion on vitamin D supplementation in this group.