CYSTIC FIBROSIS IN CHILDREN. MAIN CLINICAL ASPECTS.
Cystic fibrosis This is a severe hereditary autosomal recessive disorder associated with a mutation in the transmembrane regulator of cystic fibrosis (MVTR) located on chromosome 7, based on damage to exocrine glands and increased secretion viscosity. This often leads to damage to the lungs, pancreas and intestines, rarely the liver and kidneys. Of the five thousand orphan diseases described so far, cystic fibrosis is the most common. The frequency of meetings in the world ranges from 1: 2000 to 1: 100,000. Every year, more than 45,000 babies are born with cystic fibrosis worldwide. Frequency is relatively rare in Asian countries.
According to this study, despite significant advances in the diagnosis and treatment of cystic fibrosis, with the development of the clinical picture of the disease in the first year of life, only 18% of children under 1 year of age were diagnosed in a timely manner. One of the criteria for the severity of cystic fibrosis and its unfavorable outcome was the addition of pulmonary and biliary complications. The examined patients were diagnosed with lung atelectasis, bronchiectasis, emphysema, diffuse pneumosclerosis. This article describes a clinical case of a patient with cystic fibrosis complicated by cirrhosis of the liver, portal hypertension.
The data provided in the article will be useful for neonatologists, paediatricians, gastroenterologists and general practitioners.
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Kabdollina Zh.U., Toktabayeva B.Zh., Dolinnaya V.T., Sagyndykova G.Zh. Cystic fibrosis in children. Main clinical aspects // Nauka i Zdravookhranenie [Science & Healthcare]. 2022, (Vol.24) 3, pp. 231-237. doi 10.34689/SH.2022.24.3.027Related publications:
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