Idiopathic obliterative bronchiolitis (OB) is a rare disease that usually requires a surgical lung biopsy. A 25-year-old woman with progressive exertional dyspnea for several months showed a severe mixed restrictive and obstructive pattern on spirometry. Chest computed tomography showed a mosaic pattern, and pulmonary ventilation-perfusion scintigraphy showed a matched defect. The bronchoscopic specimens obtained from both the alveolar and bronchiolar regions of the predicted lesion area contributed to the diagnosis of OB. She had no underlying causes of secondary OB, and she was diagnosed with idiopathic OB. Since lung transplantation was indicated, she was referred to a lung transplantation-certified hospital.
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http://dx.doi.org/10.2169/internalmedicine.8490-21 | DOI Listing |
Hepatol Int
December 2024
Department of Hepatology, Institute of Liver and Biliary Sciences, New Delhi, India.
Since the Asian Pacific Association for the Study of the Liver (APASL) published guidelines on non-cirrhotic portal fibrosis/idiopathic portal hypertension in 2007, there has been a surge in new information, especially with the introduction of the term porto-sinusoidal vascular disorder (PSVD). Non-cirrhotic intra-hepatic causes of portal hypertension include disorders with a clearly identifiable etiology, such as schistosomiasis, as well as disorders with an unclear etiology such as non-cirrhotic portal fibrosis (NCPF), also termed idiopathic portal hypertension (IPH). This entity is being increasingly recognized as being associated with systemic disease and drug therapy, especially cancer therapy.
View Article and Find Full Text PDFHum Pathol
September 2024
Institute of Liver Studies, King's College Hospital, London, SE5 9RS, UK.
Since the concept of IgG4-related disease (IgG4-RD) was proposed, that diagnosis has been considered in idiopathic fibroinflammatory diseases in various organs, particularly in cases with multi-organ involvement. We have recently encountered three cases of fibrosing disease of uncertain etiology with shared microscopic appearances. Case 1 (56-year-old man) had an irregular mass at the base of mesentery.
View Article and Find Full Text PDFJ Clin Exp Hepatol
March 2024
Department of Hepatology, Kalinga Institute of Medical Sciences, Bhubaneshwar, Odisha, India.
Non-cirrhotic portal hypertension (NCPH) is a well-recognized clinico-pathological entity, which is associated with clinical signs and symptoms, imaging, and endoscopic features of portal hypertension (PHT), in absence of cirrhosis. In patients with NCPH without known risk factors of PHT or extrahepatic portal vein thrombosis, the condition is called idiopathic non-cirrhotic portal hypertension (INCPH). There are multiple infectious, immune related causes, systemic diseases, drug and toxin exposures, haematological disorders, and metabolic risk factors that have been associated with this INCPH.
View Article and Find Full Text PDFCureus
March 2024
Department of Urology, Indira Gandhi Institute of Medical Sciences, Patna, IND.
Background: Urethroplasty using a buccal mucosa graft (BMG) and a preputial skin flap (PSF) are two common techniques used for the treatment of anterior urethral stricture. The present study compared the efficacy of these two techniques on the basis of success rate, preservation of sexual function, and complications.
Materials And Methods: This prospective, randomized, interventional study was conducted on adult male patients diagnosed with non-obliterative anterior urethral strictures of length >2 cm from August 2021 to December 2022.
Am J Respir Crit Care Med
August 2024
Cardiovascular Pulmonary Research Laboratories, Department of Pediatrics and Department of Medicine.
Idiopathic pulmonary arterial hypertension (IPAH) is characterized by extensive pulmonary vascular remodeling caused by plexiform and obliterative lesions, media hypertrophy, inflammatory cell infiltration, and alterations of the adventitia. We sought to test the hypothesis that microscopic IPAH vascular lesions express unique molecular profiles, which collectively are different from control pulmonary arteries. We used digital spatial transcriptomics to profile the genomewide differential transcriptomic signature of key pathological lesions (plexiform, obliterative, intima+media hypertrophy, and adventitia) in IPAH lungs ( = 11) and compared these data with the intima+media hypertrophy and adventitia of control pulmonary artery ( = 5).
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