There are concerns about altered vascular functions that could play an important role in the pathogenesis and influence the severity of chronic disease, however, increased cardiovascular risk in paediatric cystic fibrosis (CF) has not been yet fully understood. Aim was to analyze vascular disease risk and investigate changes over times in CF and controls. We prospectively enrolled 22 CF subjects (a median age of 16.07 years), and 22 healthy demographically matched controls (a median age of 17.28 years) and determined endothelial function. We utilized a combined diagnostic approach by measuring the plethysmographic Reactive Hyperemia Index (RHI) as the post-to preocclusive endothelium-dependent changes of vascular tone, and biomarkers that are known to be related to endothelial dysfunction (ED): asymmetric dimethyl arginine (ADMA), high-sensitive CRP (hsCRP), VCAM-1 and E-selectin. RHI values were significantly lower in CF young adults (p<0.005). HsCRP (p<0.005), E-selectin (p<0.001) and VCAM-1 (p<0.001) were significantly increased in CF patients since childhood. The findings have provided a detailed account of the ongoing process of microvascular dysfunction with gradual progression with the age of CF patients, making them further at risk of advanced vascular disease. Elevations of biomarkers in CF children with not yet demonstrated RHI changes but with significantly reduced RHI in adulthood and lipid profile changes indicate the possible occurrence of ED with CF-related specific risk factors over time and will enable us to provide the best possible support.
63letý pacient, hypertonik, celoživotní kuřák, byl na kliniku TRN FN Plzeň přijat pro tumor pravé plíce zjištěný pro bolesti v pravém hemitoraxu. Na základě stážování byla stanovena diagnóza adenokarcinomu pravé plíce s generalizací do obou plic a levé nadledviny, st. IV. Byla zahájena chemoterapie Paclitaxel (200 mg/m2) – Carboplatina (AUC) – Avastin (15 mg/kg) v rámci studie SAIL firmy Roche s velmi dobrou tolerancí. Po 3. cyklu chemoterapie byla potvrzena parciální remise dle CT hrudníku, dále pokračováno ve stejném schématu chemoterapie s Avastinem do 6. cyklu. Kontrolní CT hrudníku prokázalo další regresi tumoru. Vzhledem k velmi dobrému efektu podávané léčby bylo dále pokračováno v monoterapii Avastinem v třítýdenních intervalech zatím do 22 cyklů. Po 22 měsících léčby s Avastinem bylo dosaženo trvající parciální remise nálezu dle PET/CT trupu, pacient je klinicky zcela bez potíží, neomezen v běžném životě, tedy s velmi dobrou kvalitou života (KS 90 bodů), bez vedlejších nežádoucích účinků Avastinu. Chemoterapie již nemá potenciál pro další prodlužování přežití, je tedy potřebné hledání nových léčebných strategií. Avastin jako inhibitor angiogeneze vede poprvé k prodloužení celkového přežití nad 12 měsíců. Významné prodloužení přežití bez progrese onemocnění (PFS) umožňuje delší přežití bez zhoršení symptomů., A 63-year-old hypertensive patient, a life-long smoker, presented to the Department of Tuberculosis and Respiratory Disease of the Plzeň Teaching Hospital with a right-lung tumour detected due to pain in the right hemithorax. Based on staging, a diagnosis of stage IV right lung adenocarcinoma with generalization into both lungs and the left adrenal gland was made. Chemotherapy with Paclitaxel (200 mg/m2) – Carboplatin (AUC) – Avastin (15 mg/kg) was initiated within the SAIL study of the Roche company with very good tolerance. Following the third chemotherapy cycle, partial remission was confirmed based on CT of the chest; the same chemotherapy regimen with Avastin was continued for six cycles. Follow-up CT of the chest revealed further tumour regression. Given the very good effect of the treatment administered, monotherapy with Avastin was continued in three-week intervals for 22 cycles so far. Following 22 months of Avastin treatment, sustained partial remission was achieved according to PET/CT of the trunk; the patient is clinically free of complaints, without limitations in everyday life, thus with a very good quality of life (KS of 90) and free of adverse effects of Avastin. Chemotherapy has no more potential for further extension of survival; therefore, new therapeutic strategies have to be looked for. As an angiogenesis inhibitor, Avastin leads to an extension of overall survival beyond 12 months. A significant extension of progression-free survival (PFS) enables longer survival without deterioration of symptoms., Radka Bittenglová, Miloš Pešek, and Lit.: 2