Deutsche medizinische Wochenschrift
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Dtsch. Med. Wochenschr. · Mar 2022
[Nephrotic syndrome: Current understanding and future therapies].
Advances in basic and clinical research have improved our understanding of the pathomechanisms underlying nephrotic syndrome caused by minimal change disease (MCD) and focal and segmental glomerulosclerosis (FSGS). These advances are reflected in the new 2021 KDIGO-Guidelines, which emphasize the clear distinction between primary, secondary and genetic causes. Proper classification is critical, as it directly affects the therapy of choice. ⋯ Finally, we recommend the inclusion of all MCD/FSGS patients in clinical registries (e. g. FOrMe Registry in Germany) to ensure adequate therapy and genetic testing if indicated. In addition, national registries are an invaluable source of clinical data that helps to refine our therapies towards individualized medicine.
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Dtsch. Med. Wochenschr. · Mar 2022
Case Reports[Severe lead poisoning caused by ayurvedic medicine].
We report the case of a young patient who presented to our emergency department with reduced general condition, anemia, and crampy abdominal pain. A previous inpatient workup including abdominal imaging and bone marrow aspiration had not yielded a diagnosis. On inquiry, the patient reported oral ingestion of an Ayurvedic remedy over the course of one month. ⋯ Lead has toxic effects on all organ systems of the body and is stored in the bone for decades. Symptoms of poisoning are nonspecific; a thorough history and generous indication for measuring lead levels are helpful for the diagnosis.
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Treatment of myeloproliferative neoplasia (MPN) is based on patients' individual risk-stratification and includes cytoreductive agents for high-risk essential thrombocythemia (ET), polycythemia vera (PV) and Myelofibrosis (MF). Classical cytoreductive drugs largely fail to modify the basic clonal composition of the disease. In contrast, in PV for example treatment with Ropeg-Interferon not only results in higher hematological response rates compared to hydroxyurea but in addition significantly reduces JAK2 allele-burden in high-risk PV patients as well as it depletes concurrent cytogenetic and molecular abnormalities. ⋯ Both, mutated as well as non-clonal inflammatory and other stromal cells produce significant amounts of local cytokines. Also the initiation of the neoplastic process itself seems to depend on inflammatory cytokines. Recent scRNASeq data revealed components of the alarmin complex (S100A8 und S100A9) drive this local sterile inflammation process, which also represents a potential therapeutic target, as the S100A8 and A9 inhibitor Tasquinimod reduced fibrosis in a pre-clinical animal model.
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In addition to the well-known clinical early symptoms of hantavirus disease (fever, flank and abdominal pain as well as arthralgia), unusual neurological changes in the context of infection come into focus. The spectrum of neurological symptoms ranges from transient myopia to severe pareses in the context of Guillain-Barré syndrome. In endemic areas, rapid IgM tests for initial assessment are of certain value for differential diagnosis. ⋯ A new systematic review could not confirm a human-to-human transmission previously postulated for South American hantaviruses. While all known human pathogenic hantaviruses are transmitted by rodents, other hantaviruses have been recently detected in shrews, moles, and bats. The clinical significance of these new viruses is quite unclear as yet.
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Acute kidney injury contributes significantly to morbidity and mortality in hospitalized patients and is a common complication in the intensive care unit. Identification of patients at risk, elimination of modifiable risk factors and initiation of recommended preventive measures are the main cornerstones to prevent the onset and progression of acute kidney injury. Clinical and biomarker-based risk scores can help assess AKI-risk in specific patient populations. ⋯ Nephrotoxic drugs require a critical risk-benefit assessment and therapeutic drug monitoring when appropriate. Contrast imaging should not be withheld from patients at risk of AKI when indicated but contrast medium should be limited to the smallest possible volume. Finally, recommendations include maintenance of normoglycemia and other measures to optimize organ function in specific patient populations.