| Popis: |
The article presents a complex clinical case of idiopathic recurrent pancreatitis in a young woman, 23 years old. The main clinical manifestation of the disease was acute, intense, recurrent abdominal pain. Despite ongoing symptomatic therapy, the patient’s condition had been worsened, and the intensity of abdominal pain increased. Adequate dosage of the enzyme replacement therapy allowed increasing the duration of remission. Differential diagnosis, additional laboratory and instrumental studies allowed determining the cause of recurrent pancreatitis and confirming the diagnosis of cystic fibrosis with a primary lesion of the pancreas. A distinctive feature of this clinical case is the diagnosis of a hereditary cystic fibrosis disease in adulthood, the absence of clinical symptoms of lungs’ involvement in the pathological process, as well as the absence of underdevelopment, malnutrition. The diagnosis of cystic fibrosis is confirmed by German specialists; currently, the patient continues treatment in a German clinic. The article presents current data on idiopathic pancreatitis, discusses a diagnostic algorithm for identification of the cause of the disease. The etiology, pathogenetic mechanisms of cystic fibrosis occurrence are considered, the known types of CFTR gene mutations, causing the development of the disease, as well as the principles of replacement enzyme therapy in patients with cystic fibrosis are described. Evidence-based medicine data are presented, revealing the efficacy and safety of taking enzyme preparations in the correction of the exocrine pancreatic insufficiency. |
