33 research outputs found
Π’ΡΠ°Π½ΡΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½Π½ΡΠΉ Π²Π°ΡΠΈΠ°Π½Ρ Π΄ΠΈΡΡΡΠ·Π½ΠΎΠΉ Π-ΠΊΠ»Π΅ΡΠΎΡΠ½ΠΎΠΉ ΠΊΡΡΠΏΠ½ΠΎΠΊΠ»Π΅ΡΠΎΡΠ½ΠΎΠΉ Π»ΠΈΠΌΡΠΎΠΌΡ ΠΆΠ΅Π»ΡΠ΄ΠΊΠ° Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΊΠΈ Ρ ΡΠΎΡΠ΅ΡΠ°Π½ΠΈΠ΅ΠΌ Π±ΠΎΠ»Π΅Π·Π½ΠΈ Π¨Π΅Π³ΡΠ΅Π½Π° ΠΈ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠΈ (ΠΎΠΏΠΈΡΠ°Π½ΠΈΠ΅ ΡΠ»ΡΡΠ°Ρ ΠΈ ΠΎΠ±Π·ΠΎΡ Π»ΠΈΡΠ΅ΡΠ°ΡΡΡΡ)
This article describes a case of a transformed diffuse large B-cell lymphoma of the stomach in a patient with Sjogrenβs disease (SjD) and systemic sclerosis (SSc), as well as a brief review of the literature on lymphoproliferative diseases in SjD and SSc.ΠΡΠ΅Π΄ΡΡΠ°Π²Π»Π΅Π½Ρ ΠΎΠΏΠΈΡΠ°Π½ΠΈΠ΅ ΡΠ»ΡΡΠ°Ρ ΡΡΠ°Π½ΡΡΠΎΡΠΌΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠ³ΠΎ Π²Π°ΡΠΈΠ°Π½ΡΠ° Π΄ΠΈΡΡΡΠ·Π½ΠΎΠΉ Π-ΠΊΠ»Π΅ΡΠΎΡΠ½ΠΎΠΉ ΠΊΡΡΠΏΠ½ΠΎΠΊΠ»Π΅ΡΠΎΡΠ½ΠΎΠΉ Π»ΠΈΠΌΡΠΎΠΌΡ ΠΆΠ΅Π»ΡΠ΄ΠΊΠ° Ρ ΠΏΠ°ΡΠΈΠ΅Π½ΡΠΊΠΈ Ρ Π±ΠΎΠ»Π΅Π·Π½ΡΡ Π¨Π΅Π³ΡΠ΅Π½Π° (ΠΠ¨) ΠΈ Π»ΠΈΠΌΠΈΡΠΈΡΠΎΠ²Π°Π½Π½ΠΎΠΉ ΡΠΈΡΡΠ΅ΠΌΠ½ΠΎΠΉ ΡΠΊΠ»Π΅ΡΠΎΠ΄Π΅ΡΠΌΠΈΠ΅ΠΉ (Π‘Π‘Π), Π° ΡΠ°ΠΊΠΆΠ΅ ΠΊΡΠ°ΡΠΊΠΈΠΉ ΠΎΠ±Π·ΠΎΡ Π»ΠΈΡΠ΅ΡΠ°ΡΡΡΡ, ΠΏΠΎΡΠ²ΡΡΠ΅Π½Π½ΠΎΠΉ Π»ΠΈΠΌΡΠΎΠΏΡΠΎΠ»ΠΈΡΠ΅ΡΠ°ΡΠΈΠ²Π½ΡΠΌ Π·Π°Π±ΠΎΠ»Π΅Π²Π°Π½ΠΈΡΠΌ ΠΏΡΠΈ ΠΠ¨ ΠΈ Π‘Π‘Π. ΠΠ±ΡΡΠΆΠ΄Π°ΡΡΡΡ ΡΠ²ΡΠ·ΠΈ ΠΌΠ΅ΠΆΠ΄Ρ ΡΠΊΠ°Π·Π°Π½Π½ΡΠΌΠΈ ΡΠΎΡΡΠΎΡΠ½ΠΈΡΠΌΠΈ
Diagnosis of IgG4 - related ophthalmic disease in a group of patients with various lesions of the eye and orbits
Purpose of the study. To provide demographic, clinical, laboratory, ultrasound, radiological, morphological/ immunomorphological phenotype of IgG4-related ophthalmic diseases, which allowsmaking a differential diagnosis with granulomatous, autoimmune, inflammatory, endocrine and hematologic diseases affecting the eye and orbits. Materials and methods. From 2004 to 2016 108 (78.2%) of the 138 patients were diagnosed with non-tumoral lesions of eye and orbits. In 48 patients (35%) at admission and 5 patients in the follow were diagnosed IgG4-related ophthalmic disease. In the analysis of 82 (f-44, m-38) patients with IgG4-related disease, localization of lesions in orbit observed in 53 (f-36, m-17) and it was the most frequent involvement in patients with IgG4-related disease (64.5%). Only 7 patients had isolated IgG4-related ophthalmic disease, whereas 46 patients (87%) had involvement of 2-7 locations, as a manifestation of IgG4-related systemic disease.During the examination, the average age of patients with IgG4-related ophthalmic disease was 47.5 years (19-73 years). Median time to diagnosis was 52.8 months before 2004 and 36 months 2004-2016. Results. We noted the predominance of females in the ratio 2: 1 inthe group of patients with IgG4-related ophthalmic disease. Edema of the eyelids, nasal congestion (55-60%), tumor-like formations of the upper eyelids and increased lacrimation prevailed at the onset of the disease, whereas such functional impairment like limited mobility and pain in eyeballs, exophthalmos, ptosis and diplopia appeared later at 15-38% with a loss visual acuity in one case. Bilateral lesion (86%), mainly affecting the lacrimal glands (93.5%), infiltration of the extraocular muscles (83.5%) and retrobulbar tissue with a thickening of the optic nerve in one third of patients were the main localizations IgG4-related ophthalmic disease. Clinical symptoms were accompanied by the appearance of moderate inflammatory activity (38%), increased levels IgG (44%), IgG4(88%) and IgE (61%). Indicators of autoimmune disorders observed in 6-22% of patients, most often in patients with simultaneous involvement of the salivary glands. Significant lymphoplasmacytic infiltration (94%) with a ratio of plasma cells (IgG4/IgG) secreting IgG4> 40% (90%) with fibrosis formation (94%) and follicle formation (71%) with a moderate amount of eosinophils (34%) were the major morphological / immunomorphological manifestations of IgG4-related ophthalmic disease. Signs of vasculitis and obliterative phlebitis were found in a small amount of patients. Conclusion. Determination of elevated levels of IgG-4 / IgE in patients with edema, pseudotumor of the eyelid, sinusitis and increase of the palpebral lobe of the lacrimal gland suggests the presence of IgG4-related ophthalmic disease. Minimally invasive incisional biopsy of lacrimal glands and salivary glands followed by morphological / immunomorphological research is needed for the correct diagnosis. Diagnostic orbitotomy in ophthalmic hospitals in such cases is inexpedient, since it leads to the development of dry eye. Massive lymphoplasmacytic infiltration with IgG4 / IgG ratio more than 40%, advanced fibrosis in biopsiesof the orbits tissue or salivary glands when combined lesions are required for the making the diagnosis of IgG4-related ophthalmic disease
Trans-mitochondrial coordination of cristae at regulated membrane junctions
Reminiscent of bacterial quorum sensing, mammalian mitochondria participate in inter-organelle communication. However, physical structures that enhance or enable interactions between mitochondria have not been defined. Here we report that adjacent mitochondria exhibit coordination of inner mitochondrial membrane cristae at inter-mitochondrial junctions (IMJs). These electron-dense structures are conserved across species, resistant to genetic disruption of cristae organization, dynamically modulated by mitochondrial bioenergetics, independent of known inter-mitochondrial tethering proteins mitofusins and rapidly induced by the stable rapprochement of organelles via inducible synthetic linker technology. At the associated junctions, the cristae of adjacent mitochondria form parallel arrays perpendicular to the IMJ, consistent with a role in electrochemical coupling. These IMJs and associated cristae arrays may provide the structural basis to enhance the propagation of intracellular bioenergetic and apoptotic waves through mitochondrial networks within cells