Articles Related to fibrosis

Cystic fibrosis (CF) is an inherited disorder caused by genetic mutations encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is a CFTR modulator shown to improve lung function in certain patients with CF. We undertook this study to determine its effects on glycemic outcomes in pediatric patients with cystic fibrosis-related diabetes (CFRD). We reviewed the medical records and identified two subjects, referred to as subject A and subject B, with CFRD on insulin therapy at Duke University Hospital between 2019-2020 who were on treatment with ELX/TEZ/IVA for at least one year. The mean hgbA1C pre- and post- treatment was 5.65% (5.6-5.7) and 5.05% (5.0-5.1) respectively with a mean reduction of 0.6% (p value 0.01). Improvement in hgbA1C occurred in the absence of increased insulin requirements, subject B remained on a similar insulin regimen whereas subject A was able to come off insulin completely. In addition, improvements in BMI z-score were seen in both groups with a mean BMI z-score of -0.30 pre-treatment (-0.71-0.11) and z-score of +0.28 post-treatment (0.11-0.45).

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Forty patients with CHDs who received liver biopsy to stage fibrosis and 12 normal subjects performed also shear wave elastography (SWE) and ELF-test. We chose two different outcomes for histological fibrosis (F0-F1 vs F2-F4) and (F0-F2 vs F3-F4). ELF-test and SWE were independent predictors, categorized using ROC analysis. Two scores called SCORE1 and SCORE2 were devised.

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Pseudoxanthoma elasticum (PXE) is characterized by a progressive fragmentation and calcification of elastic fibers in connective tissue and when Bruch membrane (BM) is affected ocular modifications can be found. Recently, Optical coherence tomography-angiography (OCT-A) contribute to a better comprehension of lesions in PXE.

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Infection with Pseudomonas aeruginosa in cystic fibrosis (CF) patients is a strong contributor to respiratory failure and associated mortality. Bacteria colonizing a cystic fibrosis lung commonly form biofilms that greatly contribute to increased antibiotic resistance and hypermutability. Antimicrobial treatment in these cases can be either through the use of a single agent (monotherapy) or through a combination of agents (combination therapy).

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We report here for the first time the efficacy of pegylated interferon α2a (Peg-Ifn) as a therapy for patients with myelofibrosis and high blast counts. We treated four patients who were in an accelerated phase of myeloproliferative neoplasms or acute panmyelosis with myelofibrosis using only this drug.

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Ascites due to thrombosis of the caudal vena cava is relatively seldom in cattle. To our knowledge, there have been no reports of ascites secondary to compression of the caudal vena cava by liver abscesses. This case report describes the findings in a 3.7-year-old Brown Swiss cow with this disease.

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The WHO defined JAK2V617F mutated myeloproliferative neoplasms (MPN) consist of normocellular essential thrombocythemia (ET), ET with features of early PV (prodromal PV), ET with hypercelular megakaryocytic granulocytic myeloproliferation (ET.MGM), and various stages of polycythemia vera (PV) when the WHO and European Clinical and Pathological (WHO-ECMP) criteria are applied.

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Hepatic angiosarcoma is a rare tumour that is often difficult to diagnose. Historically, most cases of hepatic angiosarcoma were seen in the setting of industrial epidemics caused by exposure of workers to toxins such as vinyl chloride. Cases associated with recognised exposure to carcinogens have fortunately been extremely rare for the last three or more decades.

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Mitogen-Activated Protein Kinase (MAPK) pathway is a signal transduction pathway that functions in a wide range of physiological and pathophysiological cellular events including cell proliferation, differentiation, apoptosis, migration, inflammation, metabolic disorders and diseases.

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