We describe a 15-year girl, who developed panhypopituitarism and diencephalic obesity after surgical excision of craniopharyngioma, followed by nonalcoholic fatty liver disease and cirrhosis 5 years after surgery. Cirrhosis in this case manifested by hypoxia due to hepatopulmonary syndrome, and despite cure of craniopharyngioma by surgery and radiosurgery treatment and adequate hormonal substitution therapy patient died 9 years after surgery. Growth hormone substitutional therapy in patients with hypopituitarism, and steatohepatitis may decrease liver triglyceride accumulation and prevent end-stage liver disease.
Diastolic myocardial dysfunction is the most important pathogenetic factor in heart failure and makes a significant contribution to hemodynamic disorders, which leads to a significant deterioration in the quality of life and prognosis of patients. This article presents a case of a patient with hypertrophic cardiomyopathy with paroxysmal atrial fibrillation and repeated pulmonary edema due to impaired diastolic function, a significant increase in mitral regurgitation and pulmonary hypertension. Given the ineffectiveness of antiarrhythmic therapy and the predicted failure of catheter ablation of the arrhythmogenic focus, the patient was scheduled for atrioventricular destruction. However, the strategy was revised to optimize the heart failure therapy, against which the tachysystole paroxysmal atrial fibrillation did not recur over the following time.
Hereditary angioedema is a rare (1:50,000) hereditary chronic disease from the group of primary immunodeficiencies, which manifests in the form of recurrent skin and mucous membrane edema of the respiratory tract and gastrointestinal tract.Pathogenesis of this disease are associated with an increased risk of a number of cardiovascular pathologies, which is associated with the complexity of management and choosing optimal treatment methods. This case report demonstrates the complexity of managing a patient with hereditary angioedema, multifocal atherosclerosis, severe aortic stenosis, and gastric neuroendocrine cancer.
Aim. To identify and study the nature of sleep-related breathing disorders (SBDs) in a cohort of hospitalized patients with heart failure (HF) with reduced and mildly reduced ejection fraction (EF), as well as to clarify the relationship between SBD type, etiology and severity of HF.Material and methods. The study included 117 patients with HF with reduced and mildly reduced ejection fraction hospitalized at the National Medical Research Center for Therapy and Preventive Medicine from 2019 to 2021. All patients underwent clinical and paraclinical examination, including cardiorespiratory sleep study. Patients were divided into three groups according to the type and severity of SBD: no or mild SBD, predominantly with obstructive sleep apnea (OSA) and predominantly with central sleep apnea (CSA). Severity of SBD and clinical data were compared between these groups.Results. A total of 5 patients (4,27%) did not have any SBDs, while 47 (40,17%) were diagnosed with CSA, and 65 (55,56%) — OSA of varying severity. The proportions of patients with moderate and severe CSA and OSA differed insignificantly and amounted to 35,9% (n=42) and 44,4% (n=52), respectively. There were following proportions of diseases related to HF: coronary artery disease (41,88%), nonischemic cardiomyopathy (26,5%), arrhythmogenic cardiomyopathy (15,38%) and other causes (16,24%) (hypertension, myocarditis, heart defects). We found that reduced EF <40%, end-diastolic volume >210 ml, and ventricular ectopy (>300 extrasystoles/day) were associated with CSA, and body mass index >30 kg/m2 was traditionally associated with OSA.Conclusion. More than half of HF patients with reduced and mildly reduced EF have SBDs. Decreased LVEF and ventricular ectopic activity are associated with CSA, while increased body mass index is associated with OSA. Consideration of SBD risk factors may improve patient phenotyping for individualized therapy.
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