Faculty of Medicine
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Item type:Publication, MECONIUM ILEUS AS INITIAL PRESENTATION OF CYSTIC FIBROSIS: A RETROSPECTIVE CASE SERIES FROM A MACEDONIAN PEDIATRIC CYSTIC FIBROSIS CENTER(University Ss. Cyril and Methodius in Skopje, 2025-12-16); Jordanova, OliveraIntroduction: Meconium ileus (MI) is often the earliest clinical sign of cystic fibrosis (CF), caused by intestinal obstruction due to thickened meconium from CFTR dysfunction. MI is commonly associated with severe CFTR mutations (classes I-III), which impair chloride and bicarbonate transport. Case report: We report a retrospective case series of six neonates with MI, diagnosed with CF at Pediatric CF Center at University Children’s Clinic, Skopje, over the last eight years. During this period, newborn bloodspot screening (NBS) program for CF was conducted, based on two sequential measurements of immunoreactive trypsinogen (IRT) and IRT-IRT protocol. All infants presented with early intestinal obstruction requiring surgical intervention, including enterotomy and ileostomy. Postoperative care included pancreatic enzyme therapy and nutritional support. CF diagnosis was confirmed via sweat chloride testing and genetic analysis, which showed a predominant presence of the F508del mutation in homozygous or compound heterozygous forms. Despite timely surgical intervention, three infants experienced severe complications and early mortality. It is noteworthy that one of these cases had false-negative NBS result, highlighting limitations of the IRT-IRT protocol in MI cases. The remaining patients showed varied recovery and nutritional outcomes. Conclusions: MI is a known cause of false-negative results in CF NBS, as IRT levels may be low in affected neonates. Therefore, any newborn presenting with MI should be presumed to have CF until proven otherwise. Confirmation requires sweat chloride testing and genetic analysis. These findings support the need for revised CF NBS protocols in all MI cases to ensure timely diagnosis and management. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Diagnosing Cystic Fibrosis in adulthood -a clinical challenge(Macedonian Medical Association = Македонско лекарско друштво, 2024-04); Ilioska Cheshlaroska, Hristina - Some of the metrics are blocked by yourconsent settings
Item type:Publication, High-resolution CT pulmonary changes in patients with cystic fibrosis using Bhalla score system correlated with clinical parameters(Macedonian Association of Anatomists, 2023-12); ; Introduction: Cystic fibrosis (CF), caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, is an inherited, chronic, progressive, and fatal disease.It mainly involves the lungs and pancreas, but also the upper airways, liver, intestine, and reproductive organs. The aim of our study is to evaluate one of the most used scoring systems, the Bhalla scoring system, in the detection of lung impairment in patients with cystic fibrosis. Material and methods: A total of 32 patients diagnosed with CF came to our University clinic of pulmonology and allergology-Skopje to perform HRCT for the first time and to detect the degree of the disease. Clinical data, age at application, deep throat and sputum cultures were obtained from medical records Moj Termin. High-resolution computed tomography (HRCT) was performed on a 128-slice PHILIPS INCISIVE CT scanner, using 1 mm slices and a high spatial resolution image reconstruction algorithm using Bhaala score system. Results: A total of 66% of patients have mild severity of bronchiectasis, 53% of all have mild peribronchial thickening. 41% of all have from 1 to 5 extent of the bronchiectasis and 53% of all have from 1 to 5 extend of mucus plugs as a dominant HRCT findings. Sputum was positive in 44 % of patients. Conclusion: High resolution computed tomography (HRCT) is well-established and is the current “gold standard” method for monitoring lung anatomical changes in patients with CF. Bhalla HRCT scoring system is useful for pulmonary evaluation of children with CF. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, Bone mineralization disorders in patients with cystic fibrosis in Republic of Macedonia(Faculty of Medicine, University Ss. Cyril and Methodius in Skopje, 2018); ; Deterioration of mineral bone density (BMD) is common in patients with cystic fibrosis (CF). It is a result of several reasons such as poor nutritional status, chronic inflammation, use of glucocorticoid therapy and others. The aim of the study was to determinate the prevalence of deterioration of mineral bone density in patients with CF by the method of absorption of X-ray dual-wavelenght (dual-energy X-ray absorptiometry-DXA) and the impact of clinical factors age, nutritional status and severity lung disease on its appearance. The study included 80 patients with CF aged 5 to 36 years who are treated at the University Children's Hospital in Skopje. Patients with CF were devided into 3 age groups: pre-puberty, from 5-11 years, puberty and adolescence 12-18 years and adults 19-36 years. All patients underwent examination of bone mineral density by DXA of the lumbal spine and some clinical parameters (body weight and height) and functional lung tests (forced expiratory volume in the first second-FEV1 and forced vital capacity-FVC). Most CF patients (68,75%) had normal mineral density (BMD); 21.25% were with osteopenia and 10% had osteoporosis. Patients with CF who have osteoporosis and osteopenia had statistically significant worse clinical parameters compared to these with normal BMD. Early detection of bone disease and prompt treatment is important in order to prevent fractures and other complications. - Some of the metrics are blocked by yourconsent settings
Item type:Publication, COPING WITH CYSTIC FIBROSIS IN THE REPUBLIC OF MACEDONIA – PARENT PERSPECTIVE(Macedonian Academy of Sciences and Arts / Sciendo, 2019); ; ; Lidija SpirevskaIntroduction: Cystic fibrosis (CF) is a progressive, life-threatening, genetic disease which mainly dam ages the lungs and the digestive system. It’s a complex medical condition, with several individual forms and variation in the symptoms severity. Few factors such as age of establishing the diagnosis, the number and the type of infections and their management, best treatment options, comorbid conditions etc. can influence the patient’s overall health, disease progression and quality of life. Many CF patients will reach adulthood, so coping with the chronic disease is very important for the overall health and everyday living. Aim of the study: To screen the quality of life in CF patients in the Republic of Macedonia, from the parent perspective. Subjects and methods: In the study we have included 55 parents of CF patients. We have created a ques tionnaire, specially designed for this survey, with questions related to their everyday coping with CF and quality of life. Results: The majority of the parents refer to the overall typical social and emotional life of their children, addressing some difficulties concerning the financial aspect of the disease and still significantly having fear from the stigma in the society. Conclusion: CF patients and their families in the Republic of Macedonia must overcome many obstacles on daily basis. Despite that, they can still have full and meaningful lives.