Terapevt Arkh
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Shwachman-Diamond syndrome is an inherited autosomal recessive disease that appears as exocrine pancreatic insufficiency, neutropenia, impaired neutrophil chemotaxis, aplastic anemia, thrombocytopenia, metaphyseal dysplasia, and physical retardation. Its worldwide prevalence is 1:10,000 to 1:20,000 live births depending on the region. ⋯ The paper describes a case of this rare disease in a 28-year-old male patient who has all characteristic manifestations as lipomatosis and severe exocrine pancreatic insufficiency, neutropenia with bone marrow hypoplasia, physical retardation, glucose intolerance, secondary osteopenia, and minor cardiac anomalies. Its clinical diagnosis was verified by molecular genetic testing.
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The paper reviews the literature on studies of the structural and functional properties of great arteries in patients with cardiovascular diseases. It analyses the pathophysiological components of the formation of central blood pressure (BP) and describes current registration procedures. The negative aspects of the impact of elevated central BP on target organs are considered. The prognostic and clinical values of the indicators of central arterial stiffness are demonstrated.
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Delirium is a neuropsychiatric condition that may complicate any visceral disease. Its rate is especially high among patients with inflammatory diseases or metabolic disturbances and in the elderly. Brain injury concurrent with an abnormal stress response underlies the development of delirium. ⋯ Special scales, such as Confusion Assessment Method for the Intensive Care Unit (CAM-ICU), are used to identify delirium. Management of delirium includes specific therapy for the underlying disease and adequate care. Low-dose neuroleptics, haloperidol in particular, are recommended to correct behavioral changes.
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Pain in patients with hemophilia is an urgent problem. The possibilities of using analgesic drugs are interesting by virtue of the specific features of pain syndrome and the problems of treatment in these patients. Foreign and Russian trials using current analgesics are analyzed; difficulties in choosing analgesic therapy and in performing anesthesia mode in patients with congenital hypocoagulation and hyperalgesia are discussed.
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Review
[Acute respiratory distress syndrome: how to optimize oxygen transport and to improve prognosis].
The paper reviews the state-of-the-art of acute respiratory distress syndrome (ARDS) and current approaches to correcting respiratory failure. It highlights the historical and present-day data on the efficiency of extracorporeal membrane hemoxygenation, high-frequency ventilation, surfactant and inhaled nitric oxide therapy, and prone ventilation. ⋯ The use of inhaled nitric oxide and surfactant achieves improved oxygenation for a while, but has no substantial effect on prognosis. The place of high-frequency oscillatory ventilation in the treatment of patients with ARDS has not been fully determined as some examinations have indicated a positive prognostic impact and other examinations have shown none or a negative impact.