Articles: treatment.
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Open spinal dysraphism is a congenital malformation that causes major morbidity. Its consequences include sensory and motor impairment as well as bladder- and bowel dysfunction. It is often also associated with prenatal ventriculomegaly, which, in turn, necessitates postnatal treatment with a ventriculoperitoneal shunt in approximately 80% of cases. Prenatal therapy with coverage of neural tube defect can reduce the shunt rate and preserve motor function. In this review, we describe the different surgical procedures and their outcomes. ⋯ Prenatal surgery improves motor function and reduces the shunt rate but long-term outcomes beyond adolescence are still lacking. Transparent and interdisciplinary counseling is essential in prenatal communication to inform parents not only about the potential benefits of this treatment, but also about its limitations and risks.
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Lung transplantation is the treatment of choice for end-stage nonmalignant lung disease. It has become a routine procedure through advances in donor lung preservation, extracorporeal membrane oxygenation, immunosuppression, intensive care medicine, and follow-up care. ⋯ Despite all the advances in lung transplantation, acute and - above all - chronic graft dysfunction still pose a major challenge for large-volume transplantation centers. Immunosuppression that is individually tailored to prevent both graft rejection and infection is important for these patients' long-term survival. Xenotransplantation and so-called lung bioengineering may become available in the future as alternatives to allotransplantation.
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Practice Guideline
The Management of Chronic Kidney Disease not Requiring Renal Replacement Therapy in General Practice.
Chronic kidney disease (CKD) is common in the German adult population, with a prevalence of 10%. This guideline, updated on the basis of current scientific evidence, contains recommendations for the management of CKD in general practice. ⋯ The risk assessment of CKD and the treatment options have been expanded. The updated guideline can improve primary care for patients with CKD and the selection of patients for interdisciplinary care.
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Polycythemia vera (PV), a myeloproliferative neoplasm characterized by an increased red blood cell mass and increased risk of thrombosis, affects approximately 65 000 people in the US, with an annual incidence of 0.5 to 4.0 cases per 100 000 persons. ⋯ PV is a myeloproliferative neoplasm characterized by erythrocytosis and is almost universally associated with a JAK2 gene variant. PV is associated with an increased risk of arterial and venous thrombosis, hemorrhage, myelofibrosis, and acute myeloid leukemia. To decrease the risk of thrombosis, all patients with PV should be treated with aspirin and therapeutic phlebotomy to maintain a hematocrit of less than 45%. Cytoreductive therapies, such as hydroxyurea or interferon, are recommended for patients at high risk of thrombosis.
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Metabolic dysfunction-associated steatotic liver disease (MASLD) is the most common chronic liver disease in the United States. It is characterized by steatosis in the liver and is potentially reversible. Risk factors include obesity, type 2 mellitus, and other metabolic disorders. ⋯ Diet, exercise, and weight loss are the cornerstones of management. Although only 1 medication has been approved for treatment of MASH, other pharmacotherapies and surgeries that aid weight loss and optimize metabolic risk factors can be used. Early diagnosis and intervention are important to prevent progression to cirrhosis and its complications, including cancer.